Abstract
The primary connective tissue diseases (also called collagen vascular diseases) are likely autoimmune with poorly understood mechanisms. This chapter reviews the non-autonomic neuropathies associated with the primary vasculitic and other connective tissue diseases. Connective tissue diseases rarely accompanied by neuropathy are not discussed (e.g., mixed connective tissue disease, relapsing polychondritis, the spondyloarthritides, dermatomyositis, Henoch-Schönlein purpura, and Behçet’s disease).
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References
Rosenbaum R. Neuromuscular complications of connective tissue diseases. Muscle Nerve. 2001;24:154–69.
Joseph A, Brasington R, Kahl L, Ranganathan P, Cheng TP, Atkinson J. Immunologic rheumatic disorders. J Allergy Clin Immunol. 2010;125(2 Suppl 2):S204–15.
Langford CA, Fauci AS. Chapter 326: The vasculitis syndromes. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison’s principles of internal medicine. 18th edn. New York: McGraw-Hill; 2012. http://www.accessmedicine.com/content.aspx?aID=9138083. Accessed 27 Dec 2011.
Miller A, Chan M, Wiik A, Misbah SA, Luqmani RA. An approach to the diagnosis and management of systemic vasculitis. Clin Exp Immunol. 2010;160:143–60.
Krishnan AV, Kiernan MC. Uremic neuropathy: clinical features and new pathophysiological insights. Muscle Nerve. 2007;35:273–90.
Dawson DM, Hallett M, Wibourn AJ. Entrapment neuropathies. 3rd ed. Philadelphia: Lippincott-Raven; 1999.
Muramatsu K, Tanaka H, Taguchi T. Peripheral neuropathies of the forearm and hand in rheumatoid arthritis: diagnosis and options for treatment. Rheumatol Int. 2008;28:951–7.
Sejvar JJ, Kohl KS, Gidudu J, Amato A, Bakshi N, Baxter R, et al. Guillain-Barré syndrome and Fisher syndrome: case definitions and guidelines for collection, analysis, and presentation of immunization safety data. Vaccine. 2011;29:599–612.
Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society – First Revisio. J Peripher Nerv Syst. 2010;15:1–9.
Florica B, Aghdassi E, Su J, Gladman DD, Urowitz MB, Fortin PR. Peripheral neuropathy in patients with systemic lupus erythematosus. Semin Arthritis Rheum. 2011;41:203–11.
Griffin JW, Cornblath DR, Alexander E, Campbell J, Low PA, Bird S, et al. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren’s syndrome. Ann Neurol. 1990;27:304–15.
De Vita S, Soldano F, Isola M, Monti G, Gabrielli A, Tzioufas A, et al. Preliminary classification criteria for the cryoglobulinaemic vasculitis. Ann Rheum Dis. 2011;70:1183–90.
Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet. 2012;379:348–60.
Charles ED, Dustin LB. Hepatitis C virus-induced cryoglobulinemia. Kidney Int. 2009;76:818–24.
Nemni R, Sanvito L, Quattrini A, Santuccio G, Camerlingo M, Canal N. Peripheral neuropathy in hepatitis C virus infection with and without cryoglobulinaemia. J Neurol Neurosurg Psychiatry. 2003;74:1267–71.
Prior R, Schober R, Scharffetter K, Wechsler W. Occlusive microangiopathy by immunoglobulin (IgM-kappa) precipitation: pathogenetic relevance in paraneoplastic cryoglobulinemic neuropathy. Acta Neuropathol. 1992;83:423–6.
Thomas FP, Lovelace RE, Ding XS, Sadiq SA, Petty GW, Sherman WH, et al. Vasculitic neuropathy in a patient with cryoglobulinemia and anti-MAG IgM monoclonal gammopathy. Muscle Nerve. 1992;15:891–8.
Bonetti B, Invernizzi F, Rizzuto N, Bonazzi ML, Zanusso GL, Chinaglia G, et al. T-cell-mediated epineurial vasculitis is and humoral-mediated microangiopathy in cryoglobulinemic neuropathy. J Neuroimmunol. 1997;73:145–54.
Stein M, Bell MJ, Ang LC. Hydroxychloroquine neuromyotoxicity. J Rheumatol. 2000;27:2927–31.
Bonnel RA, Graham DJ. Peripheral neuropathy in patients treated with leflunomide. Clin Pharmacol Ther. 2004;75:580–5.
De Weerdt A, Claeys KG, De Jonghe P, Ysebaert D, Chapelle T, Roeyen G, et al. Tacrolimus-related polyneuropathy: case report and review of the literature. Clin Neurol Neurosurg. 2008;110:291–4.
Stübgen JP. Tumor necrosis factor-alpha antagonists and neuropathy. Muscle Nerve. 2008;37:281–92.
Zara G, Ermani M, Rondinone R, Arienti S, Doria A. Thalidomide and sensory neurotoxicity: a neurophysiological study. J Neurol Neurosurg Psychiatry. 2008;79:1258–61.
Stübgen JP. Interferon alpha and neuromuscular disorders. J Neuroimmunol. 2009;212:132–41.
Santos MS, de Carvalho JF, Brotto M, Bonfa E, Rocha FA. Peripheral neuropathy in patients with primary antiphospholipid (Hughes’) syndrome. Lupus. 2010;19:583–90.
Trifiletti A, Scamardi R, Bagnato GF, Gaudio A. Hemostatic changes in vasculitides. Thromb Res. 2009;124:252–5.
Galeazzi M, Annunziata P, Sebastiani GD, Bellisai F, Campanella V, Ferrara GB, et al. Anti-ganglioside antibodies in a large cohort of European patients with systemic lupus erythematosus: clinical, serological, and HLA class II gene associations. European Concerted Action on the Immunogenetics of SLE. J Rheumatol. 2000;27:135–41.
Alpa M, Ferrero B, Cavallo R, Perna A, Naretto C, Gennaro M, et al. Anti-GM1 and anti-sulfatide antibodies in patients with systemic lupus erythematosus, Sjögren’s syndrome, mixed cryoglobulinemia and idiopathic systemic vasculitis. Clin Exp Rheumatol. 2007;25:556–62.
Basu N, Watts R, Bajema I, et al. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis. Ann Rheum Dis. 2010;69:1744–50.
Fries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. 1990;33:1135–6.
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–92.
Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222–7.
Jennette J, Falk R, Bacon P, Basu N, Cid MC, Ferrario F, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65:1–11.
Burke AP, Virmani R. Localized vasculitis. Semin Diagn Pathol. 2001;18:59–66.
Hernández-Rodríguez J, Hoffman GS. Updating single-organ vasculitis. Curr Opin Rheumatol. 2012;24:38–45.
Dyck PJ, Benstead TJ, Conn DL, Stevens JC, Windebank AJ, Low PA. Nonsystemic vasculitic neuropathy. Brain. 1987;110:843–53.
Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42:421–30.
Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62:616–26.
Dunogué B, Pagnoux C, Guillevin L. Churg-strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med. 2011;32:298–309.
Collins MP, Periquet-Collins I, Kissel JT. Vasculitis of the peripheral nervous system. In: Noseworthy JH, editor. Neurological therapeutics principles and practice, vol. 2. London: Informa; 2006. p. 2348–82.
Collins MP, Dyck PJ, Gronseth GS, Guillevin L, Hadden RD, Heuss D, et al. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst. 2010;15:176–84.
Gonzalez-Gay MA, Garcia-Porrua C. Systemic vasculitis in adults in northwestern Spain, 1988–1997. Clinical and epidemiologic aspects. Medicine. 1999;78:292–308.
Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000;43:414–9.
Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gutfleisch J, Peter HH, Raspe HH, et al. No difference in the incidences of vasculitides between north and south Germany: first results of the German vasculitis register. Rheumatology. 2002;41:540–9.
Watts RA, Scott DG. Epidemiology of the vasculitides. Semin Respir Crit Care Med. 2004;25:455–64.
Collins MP, Periquet MI. Isolated vasculitis of the peripheral nervous system. Clin Exp Rheumatol. 2008;26:S118–30.
Wees SJ, Sunwoo IN, Oh SJ. Sural nerve biopsy in systemic necrotizing vasculitis. Am J Med. 1981;71:525–32.
Said G, Lacroix-Ciaudo C, Fujimura H, Blas C, Faux N. The peripheral neuropathy of necrotizing arteritis: a clinicopathological study. Ann Neurol. 1988;23:461–5.
Hawke SH, Davies L, Pamphlett R, Guo YP, Pollard JD, McLeod JG. Vasculitic neuropathy. A clinical and pathological study. Brain. 1991;114:2175–90.
Nicolai A, Bonetti B, Lazzarino LG, Ferrari S, Monaco S, Rizzuto N. Peripheral nerve vasculitis: a clinico-pathological study. Clin Neuropathol. 1995;14:137–41.
Puéchal X, Said G, Hilliquin P, Coste J, Job-Deslandre C, Lacroix C, et al. Peripheral neuropathy with necrotizing vasculitis in rheumatoid arthritis. A clinicopathologic and prognostic study of thirty-two patients. Arthritis Rheum. 1995;38:1618–29.
Collins MP, Mendell JR, Periquet MI, Sahenk Z, Amato AA, Gronseth GS, et al. Superficial peroneal nerve/peroneus brevis muscle biopsy in vasculitic neuropathy. Neurology. 2000;55:636–43.
Hattori N, Mori K, Misu K, Koike H, Ichimura M, Sobue G. Mortality and morbidity in peripheral neuropathy associated Churg-Strauss syndrome and microscopic polyangiitis. J Rheumatol. 2002;29:1408–14.
Sugiura M, Koike H, Iijima M, Mori K, Hattori N, Katsuno M, et al. Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: a comparative study. J Neurol Sci. 2006;241:31–7.
Bennett DL, Groves M, Blake J, Holton JL, King RH, Orrell RW, et al. The use of nerve and muscle biopsy in the diagnosis of vasculitis: a 5 year retrospective study. J Neurol Neurosurg Psychiatry. 2008;79:1376–81.
Danila MI, Bridges Jr SL. Update on pathogenic mechanisms of systemic necrotizing vasculitis. Curr Rheumatol Rep. 2008;10:430–5.
Gómez-Puerta JA, Bosch X. Anti-neutrophil cytoplasmic antibody pathogenesis in small-vessel vasculitis: an update. Am J Pathol. 2009;175:1790–8.
Lepse N, Abdulahad WH, Kallenberg CG, Heeringa P. Immune regulatory mechanisms in ANCA-associated vasculitides. Autoimmun Rev. 2011;11:77–83.
Weyand CM, Younge BR, Goronzy JJ. IFN-γ and IL-17: the two faces of T-cell pathology in giant cell arteritis. Curr Opin Rheumatol. 2011;23:43–9.
Pankhurst T, Savage CO, Little MA. Review article: leukocyte-endothelial dysregulation in systemic small vessel vasculitis. Nephrology (Carlton). 2009;14:3–10.
Maugeri N, Rovere-Querini P, Baldini M, Sabbadini MG, Manfredi AA. Translational mini-review series on immunology of vascular disease: mechanisms of vascular inflammation and remodelling in systemic vasculitis. Clin Exp Immunol. 2009;156:395–404.
Muller WA. Mechanisms of leukocyte transendothelial migration. Annu Rev Pathol. 2011;6:323–44.
Hu N, Westra J, Kallenberg CG. Dysregulated neutrophil – endothelial interaction in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides: implications for pathogenesis and disease intervention. Autoimmun Rev. 2011;10:536–43.
Dale DC, Boxer L, Liles WC. The phagocytes: neutrophils and monocytes. Blood. 2008;112:935–45.
Witko-Sarsat V, Daniel S, Noël LH, Mouthon L. Neutrophils and B lymphocytes in ANCA-associated vasculitis. APMIS Suppl. 2009;117 suppl 127suppl 127:27–31.
Dustin ML, Long EO. Cytotoxic immunological synapses. Immunol Rev. 2010;235:24–34.
Maruotti N, Cantatore FP, Nico B, Vacca A, Ribatti D. Angiogenesis in vasculitides. Clin Exp Rheumatol. 2008;26:476–83.
Sprague AH, Khalil RA. Inflammatory cytokines in vascular dysfunction and vascular disease. Biochem Pharmacol. 2009;78:539–52.
Commins SP, Borish L, Steinke JW. Immunologic messenger molecules: cytokines, interferons, and chemokines. J Allergy Clin Immunol. 2010;125 Suppl 2Suppl 2:S53–72.
Zhu J, Yamane H, Paul WE. Differentiation of effector CD4 T cell populations. Annu Rev Immunol. 2010;28:445–89.
Zygmunt B, Veldhoen M. T helper cell differentiation more than just cytokines. Adv Immunol. 2011;109:159–96.
von Vietinghoff S, Ley K. Interleukin 17 in vascular inflammation. Cytokine Growth Factor Rev. 2010;21:463–9.
Nogueira E, Hamour S, Sawant D, Henderson S, Mansfield N, Chavele KM, et al. Serum IL-17 and IL-23 levels and autoantigen-specific Th17 cells are elevated in patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2010;25:2209–17.
Cochrane CG, Koffler D. Immune complex disease in experimental animals and man. Adv Immunol. 1973;16:185–264.
Jancar S, Sánchez CM. Immune complex-mediated tissue injury: a multistep paradigm. Trends Immunol. 2005;26:48–55.
Nydegger UE. Immune complex pathophysiology. Ann N Y Acad Sci. 2007;1109:66–83.
Kimberly RP. Characteristics of immune complexes and principles of immune complex diseases. In: Koopman WJ, Moreland LW, editors. Arthritis and allied conditions. 15th ed. Philadelphia: Lippincott, Williams & Wilkins; 2005.
Bosch X, Guilabert A, Font J. Antineutrophil cytoplasmic antibodies. Lancet. 2006;368:404–18.
Wiik AS. Autoantibodies in ANCA-associated vasculitis. Rheum Dis Clin North Am. 2010;36:479–89.
Flint J, Morgan MD, Savage CO. Pathogenesis of ANCA-associated vasculitis. Rheum Dis Clin North Am. 2010;36:463–77.
Jennette JC, Falk RJ, Gasim AH. Pathogenesis of antineutrophil cytoplasmic autoantibody vasculitis. Curr Opin Nephrol Hypertens. 2011;20:263–70.
Kallenberg CG. Pathogenesis of ANCA-associated vasculitis, an update. Clin Rev Allergy Immunol. 2011;41:224–31.
Mohan N, Kerr GS. ANCA-associated small vessel vasculitis: clinical and therapeutic advances. Curr Rheumatol Rep. 2010;12:406–13.
Choi HK, Liu S, Merkel PA, Colditz GA, Niles JL. Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on antimyeloperoxidase antibodies. J Rheumatol. 2001;28:1584–90.
Harper L, Cockwell P, Adu D, Savage CO. Neutrophil priming and apoptosis in anti-neutrophil cytoplasmic autoantibody-associated vasculitis. Kidney Int. 2001;59:1729–38.
Kessenbrock K, Krumbholz M, Schönermarck U, Back W, Gross WL, Werb Z, et al. Netting neutrophils in autoimmune small-vessel vasculitis. Nat Med. 2009;15:623–5.
Russell KA, Specks U. Are antineutrophil cytoplasmic antibodies pathogenic? Experimental approaches to understand the antineutrophil cytoplasmic antibody phenomenon. Rheum Dis Clin North Am. 2001;27:815–32.
Harper L, Williams JM, Savage CO. The importance of resolution of inflammation in the pathogenesis of ANCA-associated vasculitis. Biochem Soc Trans. 2004;32:502–6.
Moosig F, Csernok E, Kumanovics G, Gross WL. Opsonization of apoptotic neutrophils by anti-neutrophil cytoplasmic antibodies (ANCA) leads to enhanced uptake by macrophages and increased release of tumour necrosis factor-alpha (TNF-alpha). Clin Exp Immunol. 2000;122:499–503.
Rauova L, Gilburd B, Zurgil N, Blank M, Guegas LL, Brickman CM, et al. Induction of biologically active antineutrophil cytoplasmic antibodies by immunization with human apoptotic polymorphonuclear leukocytes. Clin Immunol. 2002;103:69–78.
Praprotnik S, Blank M, Meroni PL, Rozman B, Eldor A, Shoenfeld Y. Classification of anti-endothelial cell antibodies into antibodies against microvascular and macrovascular endothelial cells: the pathogenic and diagnostic implications. Arthritis Rheum. 2001;44:1484–94.
Meroni P, Ronda N, Raschi E, Borghi MO. Humoral autoimmunity against endothelium: theory or reality? Trends Immunol. 2005;26:275–81.
Belizna C, Duijvestijn A, Hamidou M, Tervaert JW. Antiendothelial cell antibodies in vasculitis and connective tissue disease. Ann Rheum Dis. 2006;65:1545–50.
Guilpain P, Mouthon L. Antiendothelial cells autoantibodies in vasculitis-associated systemic diseases. Clin Rev Allergy Immunol. 2008;35:59–65.
Domiciano DS, Carvalho JF, Shoenfeld Y. Pathogenic role of anti-endothelial cell antibodies in autoimmune rheumatic diseases. Lupus. 2009;18:1233–8.
Navarro M, Cervera R, Font J, Reverter JC, Monteagudo J, Escolar G, et al. Anti-endothelial cell antibodies in systemic autoimmune diseases: prevalence and clinical significance. Lupus. 1997;6:521–6.
Jamin C, Dugué C, Alard JE, Jousse S, Saraux A, Guillevin L, et al. Induction of endothelial cell apoptosis by the binding of anti-endothelial cell antibodies to Hsp60 in vasculitis-associated systemic autoimmune diseases. Arthritis Rheum. 2005;52:4028–38.
Sebastian JK, Mahr AD, Ahmed SS, Stone JH, Romay-Penabad Z, Davis JC, et al. Antiendothelial cell antibodies in patients with Wegener’s granulomatosis: prevalence and correlation with disease activity and manifestations. J Rheumatol. 2007;34:1027–31.
Brasile L, Kremer JM, Clarke JL, Cerilli J. Identification of an autoantibody to vascular endothelial cell-specific antigens in patients with systemic vasculitis. Am J Med. 1989;87:74–80.
Savage CO, Pottinger BE, Gaskin G, Lockwood CM, Pusey CD, Pearson JD. Vascular damage in Wegener’s granulomatosis and microscopic polyarteritis: presence of anti-endothelial cell antibodies and their relation to anti-neutrophil cytoplasm antibodies. Clin Exp Immunol. 1991;85:14–9.
Mayet WJ, Schwarting A, Meyer zum Büschenfelde KH. Cytotoxic effects of antibodies to proteinase 3 (C-ANCA) on human endothelial cells. Clin Exp Immunol. 1994;97:458–65.
Del Papa N, Guidali L, Sironi M, Shoenfeld Y, Mantovani A, Tincani A, et al. Anti-endothelial cell IgG antibodies from patients with Wegener’s granulomatosis bind to human endothelial cells in vitro and induce adhesion molecule expression and cytokine secretion. Arthritis Rheum. 1996;39:758–66.
Berden AE, Kallenberg CG, Savage CO, et al. Cellular immunity in Wegener’s granulomatosis: characterizing T lymphocytes. Arthritis Rheum. 2009;60:1578–87.
Hua F, Wilde B, Dolff S, Witzke O. T-lymphocytes and disease mechanisms in Wegener’s granulomatosis. Kidney Blood Press Res. 2009;32:389–98.
Zhang N, Bevan MJ. CD8(+) T cells: foot soldiers of the immune system. Immunity. 2011;35:161–8.
Andersen MH, Schrama D, Thor Straten P, Becker JC. Cytotoxic T cells. J Invest Dermatol. 2006;126:32–41.
Chávez-Galán L, Arenas-Del Angel MC, Zenteno E, Chávez R, Lascurain R. Cell death mechanisms induced by cytotoxic lymphocytes. Cell Mol Immunol. 2009;6:15–25.
Voskoboinik I, Dunstone MA, Baran K, Whisstock JC, Trapani JA. Perforin: structure, function, and role in human immunopathology. Immunol Rev. 2010;235:35–54.
Saini RV, Wilson C, Finn MW, Wang T, Krensky AM, Clayberger C. Granulysin delivered by cytotoxic cells damages endoplasmic reticulum and activates caspase-7 in target cells. J Immunol. 2011;186:3497–504.
Ewen CL, Kane KP, Bleackley RC. A quarter century of granzymes. Cell Death Differ. 2012;19:28–35.
Strasser A, Jost PJ, Nagata S. The many roles of FAS receptor signaling in the immune system. Immunity. 2009;30:180–92.
Ma-Krupa W, Jeon MS, Spoerl S, Tedder TF, Goronzy JJ, Weyand CM. Activation of arterial wall dendritic cells and breakdown of self-tolerance in giant cell arteritis. J Exp Med. 2004;199:173–83.
Ly KH, Régent A, Tamby MC, Mouthon L. Pathogenesis of giant cell arteritis: more than just an inflammatory condition? Autoimmun Rev. 2010;9:635–45.
Ciccia F, Guggino G, Rizzo A, Ferrante A, Raimondo S, Giardina A, et al. Expression of interleukin-32 in the inflamed arteries of patients with giant cell arteritis. Arthritis Rheum. 2011;63:2097–104.
Lamprecht P, Wieczorek S, Epplen JT, Ambrosch P, Kallenberg CG. Granuloma formation in ANCA-associated vasculitides. APMIS Suppl. 2009;127:32–6.
Spencer LA, Weller PF. Eosinophils and Th2 immunity: contemporary insights. Immunol Cell Biol. 2010;88:250–6.
Vaglio A, Moosig F, Zwerina J. Churg-Strauss syndrome: update on pathophysiology and treatment. Curr Opin Rheumatol. 2012;24:24–30.
Nathani N, Little MA, Kunst H, Wilson D, Thickett DR. Churg-Strauss syndrome and leukotriene antagonist use: a respiratory perspective. Thorax. 2008;63:883–8.
Kouro T, Takatsu K. IL-5- and eosinophil-mediated inflammation: from discovery to therapy. Int Immunol. 2009;21:1303–9.
Kita H. Eosinophils: multifaceted biological properties and roles in health and disease. Immunol Rev. 2011;242:161–77.
Kissel JT, Riethman JL, Omerza J, Rammohan KW, Mendell JR. Peripheral nerve vasculitis: immune characterization of the vascular lesions. Ann Neurol. 1989;25:291–7.
Satoi H, Oka N, Kawasaki T, Miyamoto K, Akiguchi I, Kimura J. Mechanisms of tissue injury in vasculitic neuropathies. Neurology. 1998;50:492–6.
Deprez M, Lubke U, Verlaet M, Debrus S, Delvenne P, Martin JJ. Detection of cytokines in human sural nerve biopsies: an immunohistochemical and molecular study. Acta Neuropathol. 2001;101:393–404.
Lindenlaub T, Sommer C. Cytokines in sural nerve biopsies from inflammatory and non-inflammatory neuropathies. Acta Neuropathol. 2003;105:593–602.
Haslbeck KM, Bierhaus A, Erwin S, Kirchner A, Nawroth P, Schlotzer U, et al. Receptor for advanced glycation endproduct (RAGE)-mediated nuclear factor-kappaB activation in vasculitic neuropathy. Muscle Nerve. 2004;29:853–60.
Kawamura N, Dyck PJ, Schmeichel AM, Engelstad JK, Low PA, Dyck PJ. Inflammatory mediators in diabetic and non-diabetic lumbosacral radiculoplexus neuropathy. Acta Neuropathol. 2008;115:231–9.
Saadoun D, Bieche I, Maisonobe T, Asselah T, Laurendeau I, Piette JC, et al. Involvement of chemokines and type 1 cytokines in the pathogenesis of hepatitis C virus-associated mixed cryoglobulinemia vasculitis neuropathy. Arthritis Rheum. 2005;52:2917–25.
Kinter J, Broglio L, Steck AJ, Tolnay M, Fuhr P, Latov N, et al. Gene expression profiling in nerve biopsy of vasculitic neuropathy. J Neuroimmunol. 2010;225:184–9.
Heuss D, Probst-Cousin S, Kayser C, Neundorfer B. Cell death in vasculitic neuropathy. Muscle Nerve. 2000;23:999–1004.
Panegyres PK, Faull RJ, Russ GR, Appleton SL, Wangel AG, Blumbergs PC. Endothelial cell activation in vasculitis of peripheral nerve and skeletal muscle. J Neurol Neurosurg Psychiatry. 1992;55:4–7.
Engelhardt A, Lorler H, Neundorfer B. Immunohistochemical findings in vasculitic neuropathies. Acta Neurol Scand. 1993;87:318–21.
Khalili-Shirazi A, Gregson NA, Londei M, Summers L, Hughes RA. The distribution of CD1 molecules in inflammatory neuropathy. J Neurol Sci. 1998;158:154–63.
Van Rhijn I, Van den Berg LH, Bosboom WM, Otten HG, Logtenberg T. Expression of accessory molecules for T-cell activation in peripheral nerve of patients with CIDP and vasculitic neuropathy. Brain. 2000;123:2020–9.
Hu W, Janke A, Ortler S, Hartung HP, Leder C, Kieseier BC, et al. Expression of CD28-related costimulatory molecule and its ligand in inflammatory neuropathies. Neurology. 2007;68:277–82.
Bosboom WM, Van den Berg LH, Mollee I, Sasker LD, Jansen J, Wokke JH, et al. Sural nerve T-cell receptor Vbeta gene utilization in chronic inflammatory demyelinating polyneuropathy and vasculitic neuropathy. Neurology. 2001;56:74–81.
Collins MP, Periquet-Collins I, Sahenk Z, Kissel JT. Direct immunofluoresence in vasculitic neuropathy: specificity of vascular immune deposits. Muscle Nerve. 2010;42:62–9.
Nukada H, Dyck PJ. Microsphere embolization of nerve capillaries and fiber degeneration. Am J Pathol. 1984;115:275–87.
Dyck PJ, Conn DL, Okazaki H. Necrotizing angiopathic neuropathy. Three-dimensional morphology of fiber degeneration related to sites of occluded vessels. Mayo Clin Proc. 1972;47:461–75.
Morozumi S, Koike H, Tomita M, Kawagashira Y, Iijima M, Katsuno M, et al. Spatial distribution of nerve fiber pathology and vasculitis in microscopic polyangiitis-associated neuropathy. J Neuropathol Exp Neurol. 2011;70:340–8.
Calabrese LH, Molloy ES, Duna G. Antineutrophil cytoplasmic antibody-associated vasculitis. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1429–51.
Cisternas M, Soto L, Jacobelli S, Marinovic MA, Vargas A, Sobarzo E, et al. Manifestaciones clinicas de la granulomatosis de Wegener y la poliangeitis microscopica en Santiago-Chile, 1990–2001. Rev Med Chil. 2005;133:273–8.
Cattaneo L, Chierici E, Pavone L, Grasselli C, Manganelli P, Buzio C, et al. Peripheral neuropathy in Wegener’s granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatry. 2007;78:1119–23.
Oh JS, Lee CK, Kim YG, Nah SS, Moon HB, Yoo B. Clinical features and outcomes of microscopic polyangiitis in Korea. J Korean Med Sci. 2009;24:269–74.
Zhang W, Zhou G, Shi Q, Zhang X, Zeng XF, Zhang FC. Clinical analysis of nervous system involvement in ANCA-associated systemic vasculitides. Clin Exp Rheumatol. 2009;27 Suppl 52Suppl 52:S65–9.
Suppiah R, Hadden RD, Batra R, Arden NK, Collins MP, Guillevin L, et al. Peripheral neuropathy in ANCA-associated vasculitis: outcomes from the European Vasculitis Study Group trials. Rheumatology. 2011;50:2214–22.
Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine. 1984;63:65–81.
Sehgal M, Swanson JW, DeRemee RA, Colby TV. Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc. 1995;70:337–41.
Solans R, Bosch JA, Pérez-Bocanegra C, Selva A, Huguet P, Alijotas J, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology. 2001;40:763–71.
Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003;115:284–90.
Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143:632–8.
Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52:2926–35.
Vinit J, Muller G, Bielefeld P, Pfitzenmeyer P, Bonniaud P, Lorcerie B, et al. Churg-Strauss syndrome: retrospective study in Burgundian population in France in past 10 years. Rheumatol Int. 2011;3:587–93.
Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993;33:4–9.
de Groot K, Schmidt DK, Arlt AC, Gross WL, Reinhold-Keller E. Standardized neurologic evaluations of 128 patients with Wegener granulomatosis. Arch Neurol. 2001;58:1215–21.
Collins MP, Periquet MI. Prevalence of vasculitic neuropathy in Wegener granulomatosis. Arch Neurol. 2002;59:1333–4.
Flores-Suárez LF, Villa AR. Spectrum of Wegener granulomatosis in a Mexican population. Ann N Y Acad Sci. 2007;1107:400–9.
Holle JU, Gross WL, Latza U, Nölle B, Ambrosch P, Heller M, et al. Improved outcome in 445 patients with Wegener’s granulomatosis in a German vasculitis center over four decades. Arthritis Rheum. 2011;63:257–66.
Hellman DB. Giant cell arteritis, polymyalgia rheumatica, and Takayasu’s arteritis. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1409–28.
Borchers AT, Gershwin ME. Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev. 2012. doi:10.1016/j.autrev.2012.01.003.
Amor-Dorado JC, Llorca J, Garcia-Porrua C, Costa C, Perez-Fernandez N, Gonzalez-Gay MA. Audiovestibular manifestations in giant cell arteritis. A prospective study. Medicine. 2003;82:13–26.
Klein RG, Hunder GG, Stanson AW, Sheps SG. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med. 1975;83:806–12.
Schmidt WA, Seifert A, Gromnica-Ihle E, Krause A, Natusch A. Ultrasound of proximal upper extremity arteries to increase the diagnostic yield in large-vessel giant cell arteritis. Rheumatology. 2008;47:96–101.
Assie C, Janvresse A, Plissonnier D, Levesque H, Marie I. Long-term follow-up of upper and lower extremity vasculitis related to giant cell arteritis: a series of 36 patients. Medicine. 2011;90:40–51.
Hayreh SS, Podhajsky PA, Raman R, Zimmerman B. Giant cell arteritis: validity and reliability of various diagnostic criteria. Am J Ophthalmol. 1997;123:285–96.
Salvarani C, Hunder GG. Giant cell arteritis with low erythrocyte sedimentation rate: frequency of occurrence in a population-based study. Arthritis Rheum. 2001;45:140–5.
Gonzalez-Gay MA, Lopez-Diaz MJ, Barros S, Garcia-Porrua C, Sanchez-Andrade A, Paz-Carreira J, et al. Giant cell arteritis: laboratory tests at the time of diagnosis in a series of 240 patients. Medicine. 2005;84:277–90.
Parikh M, Miller NR, Lee AG, Savino PJ, Vacarezza MN, Cornblath W, et al. Prevalence of a normal C-reactive protein with an elevated erythrocyte sedimentation rate in biopsy-proven giant cell arteritis. Ophthalmology. 2006;113:1842–5.
Caselli RJ, Hunder GG, Whisnant JP. Neurologic disease in biopsy-proven giant cell (temporal) arteritis. Neurology. 1988;38:352–9.
Golbus J, McCune WJ. Giant cell arteritis and peripheral neuropathy: a report of 2 cases and review of the literature. J Rheumatol. 1987;14:129–34.
Nesher G, Rosenberg P, Shorer Z, Gilai A, Solomonovich A, Sonnenblick M. Involvement of the peripheral nervous system in temporal arteritis-polymyalgia rheumatica. Report of 3 cases and review of the literature. J Rheumatol. 1987;14:358–60.
Bridges AJ, Porter J, England D. Lower extremity peripheral neuropathy and ischemic ulcers associated with giant cell arteritis. J Rheumatol. 1989;16:1366–9.
Burton EA, Winer JB, Barber PC. Giant cell arteritis of the cervical radicular vessels presenting with diaphragmatic weakness. J Neurol Neurosurg Psychiatry. 1999;67:223–6.
Nesher G. Neurologic manifestations of giant cell arteritis. Clin Exp Rheumatol. 2000;18 Suppl 20Suppl 20:S24–6.
Blaise S, Liozon E, Nadalon S, Vidal E. Maladie de Horton et plexite brachiale C5-C6: une observation avec revue de la literature. Rev Med Interne. 2005;26:578–82.
Pfadenhauer K, Roesler A, Golling A. The involvement of the peripheral nervous system in biopsy proven active giant cell arteritis. J Neurol. 2007;254:751–5.
Davies L, Spies JM, Pollard JD, McLeod JG. Vasculitis confined to peripheral nerves. Brain. 1996;119:1441–8.
Collins MP, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JT. Nonsystemic vasculitic neuropathy: insights from a clinical cohort. Neurology. 2003;61:623–30.
Kararizou E, Davaki P, Karandreas N, Davou R, Vassilopoulos D. Nonsystemic vasculitic neuropathy: a clinicopathological study of 22 cases. J Rheumatol. 2005;32:853–8.
Satoh J, Yamamoto T, Kuroda Y. Vasculitis confined to the peripheral nerve and skin: a variant of non-systemic vasculitic neuropathy. Eur J Neurol. 1999;6:241–4.
Borrie P. Cutaneous polyarteritis nodosa. Br J Dermatol. 1972;87:87–95.
Diaz-Perez JL, Winkelmann RK. Cutaneous periarteritis nodosa: a study of 33 cases. In: Wolff K, Winkelmann RK, editors. Vasculitis. London: Lloyd-Luke; 1980. p. 273–84.
Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol. 1997;136:706–13.
Nakamura T, Kanazawa N, Ikeda T, Yamamoto Y, Nakabayashi K, Ozaki S, et al. Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res. 2009;301:117–21.
Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010;49:750–6.
Ishibashi M, Chen KR. A morphological study of evolution of cutaneous polyarteritis nodosa. Am J Dermatopathol. 2008;30:319–26.
Kawakami T, Takeuchi S, Soma Y. Serum Levels of Interleukin-6 in patients with cutaneous polyarteritis nodosa. Acta Derm Venereol. 2011. doi:10.2340/00015555-1228.
Bastron JA, Thomas JE. Diabetic polyradiculopathy: clinical and electromyographic findings in 105 patients. Mayo Clin Proc. 1981;56:725–32.
Leedman PJ, Davis S, Harrison LC. Diabetic amyotrophy: reassessment of the clinical spectrum. Aust N Z J Med. 1988;18:768–73.
Barohn RJ, Sahenk Z, Warmolts JR, Mendell JR. The Bruns-Garland syndrome (diabetic amyotrophy). Revisited 100 years later. Arch Neurol. 1991;48:1130–5.
Coppack SW, Watkins PJ. The natural history of diabetic femoral neuropathy. Q J Med. 1991;79:307–13.
Said G, Goulon-Goeau C, Lacroix C, Moulonguet A. Nerve biopsy findings in different patterns of proximal diabetic neuropathy. Ann Neurol. 1994;35:559–69.
Llewelyn JG, Thomas PK, King RH. Epineurial microvasculitis in proximal diabetic neuropathy. J Neurol. 1998;245:159–65.
Younger DS, Rosoklija G, Hays AP, Trojaborg W, Latov N. Diabetic peripheral neuropathy: a clinicopathologic and immunohistochemical analysis of sural nerve biopsies. Muscle Nerve. 1996;19:722–7.
Dyck PJ, Norell JE, Dyck PJ. Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy. Neurology. 1999;53:2113–21.
Kelkar P, Masood M, Parry GJ. Distinctive pathologic findings in proximal diabetic neuropathy (diabetic amyotrophy). Neurology. 2000;55:83–8.
Garces-Sanchez M, Laughlin RS, Dyck PJ, Engelstad JK, Norell JE, Dyck PJ. Painless diabetic motor neuropathy: a variant of diabetic lumbosacral radiculoplexus neuropathy? Ann Neurol. 2011;69:1043–54.
Dyck PJ, Engelstad J, Norell J, Dyck PJ. Microvasculitis in non-diabetic lumbosacral radiculoplexus neuropathy (LSRPN): similarity to the diabetic variety (DLSRPN). J Neuropathol Exp Neurol. 2000;59:525–38.
Dyck PJ, Norell JE, Dyck PJ. Non-diabetic lumbosacral radiculoplexus neuropathy: natural history, outcome and comparison with the diabetic variety. Brain. 2001;124:1197–207.
Wartenberg R. Neuritis, sensory neuritis, neuralgia; a clinical study with review of the literature. New York: Oxford University Press; 1958. p. 233–47.
Matthews WB, Esiri M. The migrant sensory neuritis of Wartenberg. J Neurol Neurosurg Psychiatry. 1983;46:1–4.
Zifko UA, Hahn AF. Migrant sensory neuropathy: report of 5 cases and review of the literature. J Peripher Nerv Syst. 1997;2:244–9.
Simmad VI, Juel VC, Phillips LH. Clinical and electrophysiological findings in the migrant sensory ‘neuritis’ of Wartenberg. J Clin Neuromuscul Dis. 1999;1:6–10.
Nicolle MW, Barron JR, Watson BV, Hammond RR, Miller TA. Wartenberg’s migrant sensory neuritis. Muscle Nerve. 2001;24:438–43.
Stork AC, van der Meulen MF, van der Pol WL, Vrancken AF, Franssen H, Notermans NC. Wartenberg’s migrant sensory neuritis: a prospective follow-up study. J Neurol. 2010;257:1344–8.
Sobue G, Nakao N, Kumazawa K, Mitsuma T. Migrating multiple mononeuritis and nonsystemic angitis [Article in Japanese]. Rinsho Shinkeigaku. 1989;29:1210–5.
Lovshin LL, Kernohan JW. Peripheral neuritis in periarteritis nodosa. A clinicopathologic study. Arch Intern Med. 1948;82:321–8.
Moore PM, Fauci AS. Neurologic manifestations of systemic vasculitis. A retrospective and prospective study of the clinicopathologic features and responses to therapy in 25 patients. Am J Med. 1981;71:517–24.
Castaigne P, Brunet P, Hauw JJ, Léger JM, Gherardi R, Guillevin L. Systeme nerveux peripherique et panarterite noueuse. Revue de 27 cas. Rev Neurol (Paris). 1984;140:343–52.
Chang RW, Bell CL, Hallett M. Clinical characteristics and prognosis of vasculitic mononeuropathy multiplex. Arch Neurol. 1984;41:618–21.
Kissel JT, Slivka AP, Warmolts JR, Mendell JR. The clinical spectrum of necrotizing angiopathy of the peripheral nervous system. Ann Neurol. 1985;18:251–7.
Bouche P, Leger JM, Travers MA, Cathala HP, Castaigne P. Peripheral neuropathy in systemic vasculitis: clinical and electrophysiologic study of 22 patients. Neurology. 1986;36:1598–602.
Panegyres PK, Blumbergs PC, Leong AS, Bourne AJ. Vasculitis of peripheral nerve and skeletal muscle: clinicopathological correlation and immunopathic mechanisms. J Neurol Sci. 1990;100:193–202.
Singhal BS, Khadilkar SV, Gursahani RD, Surya N. Vasculitic neuropathy: profile of twenty patients. J Assoc Physicians India. 1995;43:459–61.
Chia L, Fernandez A, Lacroix C, Adams D, Plante V, Said G. Contribution of nerve biopsy findings to the diagnosis of disabling neuropathy in the elderly. A retrospective review of 100 consecutive patients. Brain. 1996;119:1091–8.
Murthy JM, Sundaram C, Meena AK. Nonsystemic vasculitic neuropathy. J Assoc Physicians India. 1998;46:204–6.
Hattori N, Ichimura M, Nagamatsu M, Li M, Yamamoto K, Kumazawa K, et al. Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. Brain. 1999;122:427–39.
Sanchez J, Coll-Canti J, Ariza A, Olive A, Cuixart A, Teixido J. Neuropathy due to necrotizing vasculitis: a study of the clinical anatomy, neurophysiological characteristics, and clinical course of the disorder in 27 patients. Rev Neurol. 2001;33:1033–6.
Seo JH, Ryan HF, Claussen GC, Thomas TD, Oh SJ. Sensory neuropathy in vasculitis: a clinical, pathologic, and electrophysiologic study. Neurology. 2004;63:874–8.
Suggs SP, Thomas TD, Joy JL, Lopez-Mendez A, Oh SJ. Vasculitic neuropathy mimicking Guillain-Barré syndrome. Arthritis Rheum. 1992;35:975–8.
Rosenbaum JT, Bell LA, Rosenbaum RB. Nonsystemic necrotizing vasculitis of the peripheral nervous system. Arthritis Rheum. 1998;41:1324–5.
Collins MP. Antineutrophil cytoplasmic antibodies in nonsystemic vasculitic neuropathy. J Rheumatol. 2007;34:899–900.
Parry GJ. Mononeuropathy multiplex (AAEE case report #11). Muscle Nerve. 1985;8:493–8.
Olney RK. AAEM minimonograph #38: neuropathies in connective tissue disease. Muscle Nerve. 1992;15:531–42.
Claussen GC, Thomas TD, Goyne C, Vazquez LG, Oh SJ. Diagnostic value of nerve and muscle biopsy in suspected vasculitis cases. J Clin Neuromuscul Dis. 2000;1:117–23.
Zivkovic SA, Ascherman D, Lacomis D. Vasculitic neuropathy – electrodiagnostic findings and association with malignancies. Acta Neurol Scand. 2007;115:432–6.
Collins MP, Periquet MI. Non-systemic vasculitic neuropathy. Curr Opin Neurol. 2004;17:587–98.
McCluskey L, Feinberg D, Cantor C, Bird S. “Pseudo-conduction block” in vasculitic neuropathy. Muscle Nerve. 1999;22:1361–6.
Sandbrink F, Klion AD, Floeter MK. “Pseudo-conduction block” in a patient with vasculitic neuropathy. Electromyogr Clin Neurophysiol. 2001;41:195–202.
Jamieson PW, Giuliani MJ, Martinez AJ. Necrotizing angiopathy presenting with multifocal conduction blocks. Neurology. 1991;41:442–4.
Magistris MR, Kohler A, Estade M. Conduction block in vasculitic neuropathy. Eur Neurol. 1994;34:283–5.
Nukada H, Dyck PJ. Acute ischemia causes axonal stasis, swelling, attenuation, and secondary demyelination. Ann Neurol. 1987;22:311–8.
Lindström P, Brismar T. Mechanism of anoxic conduction block in mammalian nerve. Acta Physiol Scand. 1991;141:429–33.
Sladky JT, Tschoepe RL, Greenberg JH, Brown MJ. Peripheral neuropathy after chronic endoneurial ischemia. Ann Neurol. 1991;29:272–8.
Nukada H, Anderson GM, McMorran PD. Reperfusion nerve injury: pathology due to reflow and prolonged ischaemia. J Peripher Nerv Syst. 1997;2:60–9.
Midroni G, Bilbao JM. Biopsy diagnosis of peripheral neuropathy. Boston: Butterworth-Heinemann; 1995. p. 241–62.
Fujimura H, Lacroix C, Said G. Vulnerability of nerve fibres to ischaemia. A quantitative light and electron microscope study. Brain. 1991;114:1929–42.
Prayson RA, Sedlock DJ. Clinicopathologic study of 43 patients with sural nerve vasculitis. Hum Pathol. 2003;34:484–90.
Vital C, Vital A, Canron MH, Jaffré A, Viallard JF, Ragnaud JM, et al. Combined nerve and muscle biopsy in the diagnosis of vasculitic neuropathy. A 16-year retrospective study of 202 cases. J Peripher Nerv Syst. 2006;11:20–9.
Said G, Lacroix C, Lozeron P, Ropert A, Plante V, Adams D. Inflammatory vasculopathy in multifocal diabetic neuropathy. Brain. 2003;126:376–85.
Vrancken AFJE, Notermans NC, Jansen GH, Wokke JHJ, Said G. Progressive idiopathic axonal neuropathy – a comparative clinical and histopathological study with vasculitic neuropathy. J Neurol. 2004;251:269–78.
Adams CW, Buk SJA, Hughes RAC, Leibowitz S, Sinclair E. Perls’ ferrocyanide test for iron in the diagnosis of vasculitic neuropathy. Neuropathol Appl Neurobiol. 1989;15:433–9.
Schenone A, De Martini I, Tabaton M, Romagnoli P, Akkad Wattar AS, Bianchini D, et al. Direct immunofluorescence in sural nerve biopsies. Eur Neurol. 1988;28:262–9.
Vrancken AF, Gathier CS, Cats EA, Notermans NC, Collins MP. The additional yield of combined nerve/muscle biopsy in vasculitic neuropathy. Eur J Neurol. 2011;18:49–58.
Deprez M, de Groote CC, Gollogly L, Reznik M, Martin JJ. Clinical and neuropathological parameters affecting the diagnostic yield of nerve biopsy. Neuromuscul Disord. 2000;10:92–8.
Schweikert K, Fuhr P, Probst A, Tolnay M, Renaud S, Steck AJ. Contribution of nerve biopsy to unclassified neuropathy. Eur Neurol. 2007;57:86–90.
Terrier B, Lacroix C, Guillevin L, Hatron PY, Dhote R, Maillot F, et al. Diagnostic and prognostic relevance of neuromuscular biopsy in primary Sjogren’s syndrome-related neuropathy. Arthritis Rheum. 2007;57:1520–9.
Chalk CH, Homburger HA, Dyck PJ. Anti-neutrophil cytoplasmic ant. bodies in vasculitis peripheral neuropathy. Neurology. 1993;43:1826–7.
Sakai K, Komai K, Yanase D, Yamada M. Plasma VEGF as a marker for the diagnosis and treatment of vasculitic neuropathy. J Neurol Neurosurg Psychiatry. 2005;76:296.
Guillevin L, Mahr A, Callard P, Godmer P, Pagnoux C, Leray E, et al. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine. 2005;84:313–22.
Saadoun D, Resche-Rigon M, Thibault V, Piette JC, Cacoub P. Antiviral therapy for hepatitis C virus – associated mixed cryoglobulinemia vasculitis: a long-term followup study. Arthritis Rheum. 2006;54:3696–706.
Gisselbrecht M, Cohen P, Lortholary O, Jarrousse B, Gayraud M, Ghérardi R, et al. HIV-related vasculitis: clinical presentation and therapeutic approach on six patients. AIDS. 1997;11:121–3.
Halperin JJ. Nervous system Lyme disease: is there a controversy? Semin Neurol. 2011;31:317–24.
Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98:76–85.
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488–98.
Reinhold-Keller E, Beuge N, Latza U, de Groot K, Rudert H, Nölle B, et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000;43:1021–32.
Knight A, Askling J, Ekbom A. Cancer incidence in a population-based cohort of patients with Wegener’s granulomatosis. Int J Cancer. 2002;100:82–5.
Faurschou M, Sorensen IJ, Mellemkjaer L, Loft AG, Thomsen BS, Tvede N, et al. Malignancies in Wegener’s granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J Rheumatol. 2008;35:100–5.
Lapraik C, Watts R, Bacon P, Carruthers D, Chakravarty K, D’Cruz D, et al. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology. 2007;46:1615–6.
Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. EULAR Recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68:310–7.
Walsh M, Merkel PA, Mahr A, Jayne D. Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: a meta-analysis. Arthritis Care Res. 2010;62:1166–73.
Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790–803.
de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150:670–80.
Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA. 2010;304:2381–8.
Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007;18:2180–8.
Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363:211–20.
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363:221–32.
Falk RJ, Jennette JC. Rituximab in ANCA-associated disease. N Engl J Med. 2010;363:285–6.
Ntatsaki E, Mooney J, Watts RA. ANCA vasculitis: time for a change in treatment paradigm? Not yet. Rheumatology. 2011;50:1019–24.
Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G. Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome. Ann Rheum Dis. 2004;63:1649–54.
Jayne DR, Chapel H, Adu D, Misbah S, O’Donoghue D, Scott D, et al. Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM. 2000;93:433–9.
Finnerup NB, Sindrup SH, Jensen TS. The evidence for pharmacological treatment of neuropathic pain. Pain. 2010;150:573–5.
Hajj-Ali RA, Wilke WS, Calabrese LH, Hoffman GS, Liu X, Bena J, et al. Pilot study to assess the frequency of fibromyalgia, depression, and sleep disorders in patients with granulomatosis with polyangiitis (Wegener’s). Arthritis Care Res. 2011;63:827–33.
Kenna HA, Poon AW, de los Angeles CP, Koran LM. Psychiatric complications of treatment with corticosteroids: review with case report. Psychiatry Clin Neurosci. 2011;65:549–60.
Boyd LJ. IV. Periarteritis nodosa. Neuromyositic manifestations. NY Med Coll Flow Hosp Bull. 1940;3:272–9.
Kernohan JW, Woltman HW. Periarteritis nodosa: a clinicopathological study with special reference to the nervous system. Arch Neurol Psychiatry. 1938;39:655–86.
Puechal X, Said G. Necrotizing vasculitis of the peripheral nervous system: nonsystemic or clinically undetectable? Arthritis Rheum. 1999;42:824–5.
Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S94–104.
Scott DL, Wolfe F, Huizinga TW. Rheumatoid arthritis. Lancet. 2010;376:1094–108.
Shaw A, Clair EW. Chapter 321: Rheumatoid arthritis. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison’s principles of internal medicine. 18th ed. New York: McGraw-Hill; 2012. http://www.accessmedicine.com/content.aspx?aID=9136970. Accessed 27 Dec 2011.
Alamanos Y, Voulgari PV, Drosos AA. Incidence and prevalence of rheumatoid arthritis, based on the 1987 American College of Rheumatology criteria: a systematic review. Semin Arthritis Rheum. 2006;36:182–8.
Firestein GS. Etiology and pathogenesis of rheumatoid arthritis. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1035–86.
Imboden JB. The immunopathogenesis of rheumatoid arthritis. Annu Rev Pathol. 2009;4:417–34.
Cooles FA, Isaacs JD. Pathophysiology of rheumatoid arthritis. Curr Opin Rheumatol. 2011;23:233–40.
McInnes IB, Schett G. The pathogenesis of rheumatoid arthritis. N Engl J Med. 2011;365:2205–19.
de Vries RR, van der Woude D, Houwing JJ, Toes RE. Genetics of ACPA-positive rheumatoid arthritis: the beginning of the end? Ann Rheum Dis. 2011;70 Suppl 1Suppl 1:i51–4.
Perricone C, Ceccarelli F, Valesini G. An overview on the genetic of rheumatoid arthritis: a never-ending story. Autoimmun Rev. 2011;10:599–608.
Bax M, van Heemst J, Huizinga TW, Toes RE. Genetics of rheumatoid arthritis: what have we learned? Immunogenetics. 2011;63:459–66.
Wegner N, Lundberg K, Kinloch A, Fisher B, Malmström V, Feldmann M, et al. Autoimmunity to specific citrullinated proteins gives the first clues to the etiology of rheumatoid arthritis. Immunol Rev. 2010;233:34–54.
Quirke AM, Fisher BA, Kinloch AJ, Venables PJ. Citrullination of autoantigens: upstream of TNFα in the pathogenesis of rheumatoid arthritis. FEBS Lett. 2011;585:3681–8.
van Venrooij WJ, van Beers JJ, Pruijn GJ. Anti-CCP antibodies: the past, the present and the future. Nat Rev Rheumatol. 2011;7:391–8.
Hale LP. Pathology of rheumatoid arthritis and associated disorders. In: Koopman WJ, Moreland LW, editors. Arthritis and allied conditions. 15th ed. Philadelphia: Lippincott, Williams & Wilkins; 2005.
Li X, Yuan FL, Lu WG, Zhao YQ, Li CW, Li JP, et al. The role of interleukin-17 in mediating joint destruction in rheumatoid arthritis. Biochem Biophys Res Commun. 2010;397:131–5.
Ballanti E, Perricone C, di Muzio G, Kroegler B, Chimenti MS, Graceffa D, et al. Role of the complement system in rheumatoid arthritis and psoriatic arthritis: relationship with anti-TNF inhibitors. Autoimmun Rev. 2011;10:617–23.
Bartok B, Firestein GS. Fibroblast-like synoviocytes: key effector cells in rheumatoid arthritis. Immunol Rev. 2010;233:233–55.
Karmakar S, Kay J, Gravallese EM. Bone damage in rheumatoid arthritis: mechanistic insights and approaches to prevention. Rheum Dis Clin North Am. 2010;36:385–404.
van der Zee JM, Heurkens AH, van der Voort EA, Daha MR, Breedveld FC. Characterization of anti-endothelial antibodies in patients with rheumatoid arthritis complicated by vasculitis. Clin Exp Rheumatol. 1991;9:589–94.
Guillevin L, Dörner T. Vasculitis: mechanisms involved and clinical manifestations. Arthritis Res Ther. 2007;9 Suppl 2:S9.
Tihlirian CV, Bathon JM. Rheumatoid arthritis. A. Clinical and laboratory manifestations. In: Klippel JH, Stone JH, Crofford LJ, White PH, editors. Primer on the rheumatic disease. 13th ed. New York: Springer; 2008. p. 114–21.
Harris ED, Firestein GS. Clinical features of rheumatoid arthritis. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1087–118.
Mielants H, Van den Bosch F. Extra-articular manifestations. Clin Exp Rheumatol. 2009;27 Suppl 55Suppl 55:S56–61.
Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases: a meta-analysis. Arch Intern Med. 2005;165:2337–44.
Pieringer H, Pichler M. Cardiovascular morbidity and mortality in patients with rheumatoid arthritis: vascular alterations and possible clinical implications. QJM. 2011;104:13–26.
Scott DG, Bacon PA, Tribe CR. Systemic rheumatoid vasculitis: a clinical and laboratory study of 50 cases. Medicine. 1981;60:288–97.
Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006;36:88–98.
Turesson C, Matteson EL. Vasculitis in rheumatoid arthritis. Curr Opin Rheumatol. 2009;21:35–40.
Bartels CM, Bridges AJ. Rheumatoid vasculitis: vanishing menace or target for new treatments? Curr Rheumatol Rep. 2010;12:414–9.
Cruickshank B. The arteritis of rheumatoid arthritis. Ann Rheum Dis. 1954;13:136–46.
Kemper JW, Baggenstoss AH, Slocumb CH. The relationship of therapy with cortisone to the incidence of vascular lesions in rheumatoid arthritis. Ann Intern Med. 1957;46:831–51.
Boers M, Croonen AM, Dijkmans BA, Breedveld FC, Eulderink F, Cats A, et al. Renal findings in rheumatoid arthritis: clinical aspects of 132 necropsies. Ann Rheum Dis. 1987;46:658–63.
Suzuki A, Ohosone Y, Obana M, Mita S, Matsuoka Y, Irimajiri S, et al. Cause of death in 81 autopsied patients with rheumatoid arthritis. J Rheumatol. 1994;21:33–6.
Bely M, Apathy A. Vasculitis rheumatoid arthritisben. Orv Hetil. 1996;137:15711578.
Watts RA, Mooney J, Lane SE, Scott DG. Rheumatoid vasculitis: becoming extinct? Rheumatology. 2004;43:920–3.
Bartels C, Bell C, Rosenthal A, Shinki K, Bridges A. Decline in rheumatoid vasculitis prevalence among US veterans: a retrospective cross-sectional study. Arthritis Rheum. 2009;60:2553–7.
Myasoedova E, Crowson CS, Turesson C, Gabriel SE, Matteson EL. Incidence of extraarticular rheumatoid arthritis in Olmsted County, Minnesota, in 1995–2007 versus 1985–1994: a population-based study. J Rheumatol. 2011;38:983–9.
Schmid FR, Cooper NS, Ziff M, McEwen C. Arteritis in rheumatoid arthritis. Am J Med. 1961;30:56–83.
Vollertsen RS, Conn DL, Ballard DJ, Ilstrup DM, Kazmar RE, Silverfield JC. Rheumatoid vasculitis: survival and associated risk factors. Medicine. 1986;65:365–75.
Voskuyl AE, Zwinderman AH, Westedt ML, Vandenbroucke JP, Breedveld FC, Hazes JM. Factors associated with the development of vasculitis in rheumatoid arthritis: results of a case–control study. Ann Rheum Dis. 1996;55:190–2.
Laskari K, Ahmadi-Simab K, Lamken M, Csernok E, Gross WL, Hellmich B. Are anti-cyclic citrullinated peptide autoantibodies seromarkers for rheumatoid vasculitis in a cohort of patients with systemic vasculitis? Ann Rheum Dis. 2010;69:469–71.
Arnett FC, Edworthy SM, Bloch DA, McShane DJ, Fries JF, Cooper NS, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum. 1988;31:315–24.
Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO, et al. 2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2010;62:2569–81.
Tur BS, Süldür N, Ataman S, Tutkak H, Atay MB, Düzgün N. Anti-neutrophil cytoplasmic antibodies in patients with rheumatoid arthritis: clinical, biological, and radiological correlations. Joint Bone Spine. 2004;71:198–202.
Kida I, Kobayashi S, Takeuchi K, Tsuda H, Hashimoto H, Takasaki Y. Antineutrophil cytoplasmic antibodies against myeloperoxidase, proteinase 3, elastase, cathepsin G and lactoferrin in Japanese patients with rheumatoid arthritis. Mod Rheumatol. 2011;21:43–50.
Whiting PF, Smidt N, Sterne JA, Harbord R, Burton A, Burke M, et al. Systematic review: accuracy of anti-citrullinated peptide antibodies for diagnosing rheumatoid arthritis. Ann Intern Med. 2010;152:456–64.
Dörner T, Egerer K, Feist E, Burmester GR. Rheumatoid factor revisited. Curr Opin Rheumatol. 2004;16:246–53.
Goodyear CS, Tighe H, McInnes IB. Rheumatoid factors and other autoantibodies in rheumatoid arthritis. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 755–65.
Rantapää-Dahlqvist S, de Jong BA, Berglin E, Hallmans G, Wadell G, Stenlund H, et al. Antibodies against cyclic citrullinated peptide and IgA rheumatoid factor predict the development of rheumatoid arthritis. Arthritis Rheum. 2003;48:2741–9.
Aggarwal R, Liao K, Nair R, Ringold S, Costenbader KH. Anti-citrullinated peptide antibody assays and their role in the diagnosis of rheumatoid arthritis. Arthritis Rheum. 2009;15(61):1472–83.
Genovese MC. Treatment of rheumatoid arthritis. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1119–43.
McInnes IB, O’Dell JR. State-of-the-art: rheumatoid arthritis. Ann Rheum Dis. 2010;69:1898–906.
Finckh A, Liang MH, van Herckenrode CM, de Pablo P. Long-term impact of early treatment on radiographic progression in rheumatoid arthritis: a meta-analysis. Arthritis Rheum. 2006;55:864–72.
Haraoui B, Pope J. Treatment of early rheumatoid arthritis: concepts in management. Semin Arthritis Rheum. 2011;40:371–88.
Saag KG, Teng GG, Patkar NM, Anuntiyo J, Finney C, Curtis JR, et al. American College of Rheumatology 2008 recommendations for the use of nonbiologic and biologic disease-modifying antirheumatic drugs in rheumatoid arthritis. Arthritis Rheum. 2008;59:762–84.
Smolen JS, Landewé R, Breedveld FC, Dougados M, Emery P, Gaujoux-Viala C, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs. Ann Rheum Dis. 2010;69:964–75.
Colmegna I, Ohata BR, Menard HA. Current understanding of rheumatoid arthritis therapy. Clin Pharmacol Ther. 2012. doi:10.1038/clpt.2011.325.
Aaltonen KJ, Virkki LM, Malmivaara A, Konttinen YT, Nordström DC, Blom M. Systematic review and meta-analysis of the efficacy and safety of existing TNF blocking agents in treatment of rheumatoid arthritis. PLoS One. 2012;7:e30275.
Scott DL. Biologics-based therapy for the treatment of rheumatoid arthritis. Clin Pharmacol Ther. 2012;91:30–43.
Gorter SL, Bijlsma JW, Cutolo M, Gomez-Reino J, Kouloumas M, Smolen JS, et al. Current evidence for the management of rheumatoid arthritis with glucocorticoids: a systematic literature review informing the EULAR recommendations for the management of rheumatoid arthritis. Ann Rheum Dis. 2010;69:1010–4.
Luqmani RA, Pathare S, Kwok-Fai TL. How to diagnose and treat secondary forms of vasculitis. Best Pract Res Clin Rheumatol. 2005;19:321–36.
Scott DG, Bacon PA. Intravenous cyclophosphamide plus methylprednisolone in treatment of systemic rheumatoid vasculitis. Am J Med. 1984;76:377–84.
Rell-Bakalarska M, Filipowicz-Sosnowska A, Garwolinska H, Zabek J. Assessment of the results of combined treatment with cyclophosphamide and prednisone or methotrexate and prednisone in patients with rheumatoid arthritis with concomitant vasculitis [Polish]. Rhematologia. 1998;36:15–22.
Puéchal X, Miceli-Richard C, Mejjad O, Lafforgue P, Marcelli C, Solau-Gervais E, et al. Anti-tumour necrosis factor treatment in patients with refractory systemic vasculitis associated with rheumatoid arthritis. Ann Rheum Dis. 2008;67:880–4.
Puéchal X, Gottenberg JE, Berthelot JM, Gossec L, Meyer O, Morel J, et al. Rituximab therapy for systemic vasculitis associated with rheumatoid arthritis: results from the autoimmunity and rituximab registry. Arthritis Care Res. 2012;64:331–9.
Fujii W, Kohno M, Ishino H, Nakabayashi A, Fujioka K, Kida T, et al. The rapid efficacy of abatacept in a patient with rheumatoid vasculitis. Mod Rheumatol. 2011. doi:10.1007/s10165-011-0559-8.
Sumida K, Ubara Y, Takemoto F, Takaichi K. Successful treatment with humanised anti-interleukin 6 receptor antibody for multidrug-refractory and anti-tumour necrosis factor-resistant systemic rheumatoid vasculitis. Clin Exp Rheumatol. 2011;29(1 Suppl 64):S133.
Machold KP, Stamm TA, Nell VP, Pflugbeil S, Aletaha D, Steiner G, et al. Very recent onset rheumatoid arthritis: clinical and serological patient characteristics associated with radiographic progression over the first years of disease. Rheumatology. 2007;46:342–9.
Scott DL, Steer S. The course of established rheumatoid arthritis. Best Pract Res Clin Rheumatol. 2007;21:943–67.
Sokka T, Abelson B, Pincus T. Mortality in rheumatoid arthritis: 2008 update. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S35–61.
Myasoedova E, Davis 3rd JM, Crowson CS, Gabriel SE. Epidemiology of rheumatoid arthritis: rheumatoid arthritis and mortality. Curr Rheumatol Rep. 2010;12:379–85.
Bharadwaj A, Haroon N. Interstitial lung disease and neuropathy as predominant extra-articular manifestations in patients with rheumatoid arthritis: a prospective study. Med Sci Monit. 2005;11:CR498–502.
Sakini RA, Abdul-Zehra IK, Al-Nimer MS. Neuropathic manifestations in rheumatoid arthritis: a clinical and electrophysiological assessment in a small sample of Iraqi patients. Ann Saudi Med. 2005;25:247–9.
Albani G, Ravaglia S, Cavagna L, Caporali R, Montecucco C, Mauro A. Clinical and electrophysiological evaluation of peripheral neuropathy in rheumatoid arthritis. J Peripher Nerv Syst. 2006;11:174–5.
Agarwal V, Singh R, Wiclaf, Chauhan S, Tahlan A, Ahuja CK, et al. A clinical, electrophysiological, and pathological study of neuropathy in rheumatoid arthritis. Clin Rheumatol. 2008;27:841–4.
Corona-Sanchez EG, Gonzalez-Lopez L, Muñoz-Valle JF, Vazquez-Del Mercado M, Lopez-Olivo MA, Aguilar-Chavez EA, et al. Circulating E-selectin and tumor necrosis factor-alpha in extraarticular involvement and joint disease activity in rheumatoid arthritis. Rheumatol Int. 2009;29:281–6.
Bayrak AO, Durmus D, Durmaz Y, Demir I, Canturk F, Onar MK. Electrophysiological assessment of polyneuropathic involvement in rheumatoid arthritis: relationships among demographic, clinical and laboratory findings. Neurol Res. 2010;32:711–4.
Lanzillo B, Pappone N, Crisci C, di Girolamo C, Massini R, Caruso G. Subclinical peripheral nerve involvement in patients with rheumatoid arthritis. Arthritis Rheum. 1998;41:1196–202.
Geirsson AJ, Sturfelt G, Truedsson L. Clinical and serological features of severe vasculitis in rheumatoid arthritis: prognostic implications. Ann Rheum Dis. 1987;46:727–33.
Wattiaux MJ, Kahn MF, Thevenet JP, Sauvezie B, Imbert JC. L’atteinte vasculaire dans la polyarthrite rhumatoide. Etude retrospective de 37 polyarthrites rhumatoides avec atteinte vasculaire et revue de la litterature. Ann Med Interne (Paris). 1987;138:566–87.
Schneider HA, Yonker RA, Katz P, Longley S, Panush RS. Rheumatoid vasculitis: experience with 13 patients and review of the literature. Semin Arthritis Rheum. 1985;14:280–6.
Heurkens AH, Westedt ML, Breedveld FC. Prednisone plus azathioprine treatment in patients with rheumatoid arthritis complicated by vasculitis. Arch Intern Med. 1991;151:2249–54.
Hart FD, Golding JR. Rheumatoid neuropathy. Br Med J. 1960;1(5186):1594–602.
Pallis CA, Scott JT. Peripheral neuropathy in rheumatoid arthritis. Br Med J. 1965;1(5443):1141–7.
Haslock DI, Wright V, Harriman DG. Neuromuscular disorders in rheumatoid arthritis. A motor-point muscle biopsy study. Q J Med. 1970;39:335–58.
Weller RO, Bruckner FE, Chamberlain MA. Rheumatoid neuropathy: a histological and electrophysiological study. J Neurol Neurosurg Psychiatry. 1970;33:592–604.
Beckett VL, Dinn JJ. Segmental demyelination in rheumatoid arthritis. Q J Med. 1972;41:71–80.
Conn DL, McDuffie FC. The pathogenesis of rheumatoid neuropathy. In: Eberl R, Rosenthal M, chairmen. Organic manifestations and complications in rheumatoid arthritis. Symposium Hernstein, 2nd–5th Nov 1975. New York: FK Schattauer Verlag; 1976. p 295–306.
Kobak S. Demographic, clinical, and serological features of Turkish patients with rheumatoid arthritis: evaluation of 165 patients. Clin Rheumatol. 2011;30:843–7.
Karadag O, Kalyoncu U, Akdogan A, Karadag YS, Bilgen SA, Ozbakır S, et al. Sonographic assessment of carpal tunnel syndrome in rheumatoid arthritis: prevalence and correlation with disease activity. Rheumatol Int. 2011. doi:10.1007/s00296-011-1957-0.
Hanna BD, Robertson FW, Ansell BM, Maudsley RH. Nerve entrapment at the elbow in rheumatoid arthritis. Rheumatol Rehabil. 1975;14:212–7.
Balagtas-Balmaseda OM, Grabois M, Balmaseda PF, Lidsky MD. Cubital tunnel syndrome in rheumatoid arthritis. Arch Phys Med Rehabil. 1983;64:163–6.
Malipeddi A, Reddy VR, Kallarackal G. Posterior interosseous nerve palsy: an unusual complication of rheumatoid arthritis: case report and review of the literature. Semin Arthritis Rheum. 2011;40:576–9.
Nakano KK. The entrapment neuropathies of rheumatoid arthritis. Orthop Clin North Am. 1975;6:837–60.
Ishikawa H, Hirohata K. Bilateral peroneal nerve palsy secondary to posterior dislocation of the proximal tibiofibular joint in rheumatoid arthritis. Rheumatol Int. 1984;5:45–7.
LeTourneau L, Dessureault M, Carette S. Rheumatoid iliopsoas bursitis presenting as unilateral femoral nerve palsy. J Rheumatol. 1991;18:462–3.
Vilches A, Burke MJ. Trigeminal sensory neuropathy and rheumatoid arthritis: case study of a rare association. Rheumatology. 2001;40:832–3.
Hirohashi N, Sakai T, Sairyo K, Oba K, Higashino K, Katoh S, et al. Lumbar radiculopathy caused by extradural rheumatoid nodules. Case report. J Neurosurg Spine. 2007;7:352–6.
Markenson JA, McDougal JS, Tsairis P, Lockshin MD, Christian CL. Rheumatoid meningitis: a localized immune process. Ann Intern Med. 1979;90:786–9.
White KP, Harth M. Lumbar pannus presenting as cauda equina syndrome in a patient with longstanding rheumatoid arthritis. J Rheumatol. 2001;28:627–30.
Kawaji H, Miyamoto M, Gembun Y, Ito H. A case report of rapidly progressing cauda equina symptoms due to rheumatoid arthritis. J Nihon Med Sch. 2005;72:290–4.
Nakamura T. Amyloid A, amyloidosis secondary to rheumatoid arthritis: pathophysiology and treatments. Clin Exp Rheumatol. 2011;29:850–7.
Obici L, Raimondi S, Lavatelli F, Bellotti V, Merlini G. Susceptibility to AA amyloidosis in rheumatic diseases: a critical overview. Arthritis Rheum. 2009;61:1435–40.
Browning MJ, Banks RA, Tribe CR, Hollingworth P, Kingswood C, Mackenzie JC, et al. Ten years’ experience of an amyloid clinic – a clinicopathological survey. Q J Med. 1985;54:213–27.
Tsunoda I, Awano H, Kayama H, Tsukamoto T, Ueno S, Fujiwara T, et al. Idiopathic AA amyloidosis manifested by autonomic neuropathy, vestibulocochleopathy, and lattice corneal dystrophy. J Neurol Neurosurg Psychiatry. 1994;57:635–7.
Tziofas AG, Mitsias DI, Monsopoulos HM. Sjögren’s syndrome. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, editors. Rheumatology. 4th ed. Philadelphia: Mosby/Elsevier; 2007. p. 1342–52.
Ng KP, Isenberg DA. Sjögren’s syndrome. Diagnosis and therapeutic challenges in the elderly. Drugs Aging. 2008;25:19–33.
Carsons S. Sjögren syndrome. In: Firestein GS, Budd RC, Harris ED, McInnes IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1149–68.
Binard A, Devauchelle-Pensec V, Fautrel B, Jousse S, Youinou P, Saraux A. Epidemiology of Sjögren’s syndrome: where are we now? Clin Exp Rheumatol. 2007;25:1–4.
Gøransson LG, Haldorsen K, Brun JG, Harboe E, Jonsson MV, Skarstein K, et al. The point prevalence of clinically relevant primary Sjögren’s syndrome in two Norwegian counties. Scand J Rheumatol. 2011;40:221–4.
Daniels T. Sjögren’s syndrome. In: Klippel JH, Stone JH, Crofford LJ, White PH, editors. Primer on the rheumatic disease. 13th ed. New York: Springer; 2008. p. 389–97.
Mariette X, Gottenberg JE. Pathogenesis of Sjögren’s syndrome and therapeutic consequences. Curr Opin Rheumatol. 2010;22:471–7.
Voulgarelis M, Tzioufas AG. Pathogenetic mechanisms in the initiation and perpetuation of Sjögren’s syndrome. Nat Rev Rheumatol. 2010;6:529–37.
Mitsias DI, Kapsogeorgou EK, Moutsopoulos HM. The role of epithelial cells in the initiation and perpetuation of autoimmune lesions: lessons from Sjogren’s syndrome (autoimmune epithelitis). Lupus. 2006;15:255–61.
Manoussakis MN, Kapsogeorgou EK. The role of intrinsic epithelial activation in the pathogenesis of Sjögren’s syndrome. J Autoimmun. 2010;35:219–24.
Cobb BL, Lessard CJ, Harley JB, Moser KL. Genes and Sjögren’s syndrome. Rheum Dis Clin North Am. 2008;34:847–68.
Ice JA, Li H, Adrianto I, Lin PC, Kelly JA, Montgomery CG, et al. Genetics of Sjögren’s syndrome in the genome-wide association era. J Autoimmun. 2012. doi:10.1016/j.jaut.2012.01.008.
Katsifis GE, Rekka S, Moutsopoulos NM, Pillemer S, Wahl SM. Systemic and local interleukin-17 and linked cytokines associated with Sjögren’s syndrome immunopathogenesis. Am J Pathol. 2009;175:1167–77.
Bombardieri M, Pitzalis C. Ectopic lymphoid neogenesis and lymphoid chemokines in Sjogren’s syndrome: at the interplay between chronic inflammation, autoimmunity and lymphomagenesis. Curr Pharm Biotechnol. 2012;13(10):1989–96.
Youinou P, Devauchelle-Pensec V, Pers JO. Significance of B cells and B cell clonality in Sjögren’s syndrome. Arthritis Rheum. 2010;62:2605–10.
Routsias JG, Tzioufas AG. Sjögren’s syndrome – study of autoantigens and autoantibodies. Clin Rev Allergy Immunol. 2007;32:238–51.
Mavragani CP, Crow MK. Activation of the type I interferon pathway in primary Sjogren’s syndrome. J Autoimmun. 2010;35:225–31.
Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum. 2000;29:296–304.
Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA. Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology. 2006;45:187–91.
Atzeni F, Sarzi-Puttini P, Lama N, Bonacci E, Bobbio-Pallavicini F, Montecucco C, et al. Anti-cyclic citrullinated peptide antibodies in primary Sjögren syndrome may be associated with non-erosive synovitis. Arthritis Res Ther. 2008;10:R51.
Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, et al. Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine. 2008;87:210–9.
Fauchais AL, Martel C, Gondran G, Lambert M, Launay D, Jauberteau MO, et al. Immunological profile in primary Sjögren syndrome: clinical significance, prognosis and long-term evolution to other auto-immune disease. Autoimmun Rev. 2010;9:595–9.
Malladi AS, Sack KE, Shiboski S, Shiboski C, Baer AN, Banushree R, et al. Primary Sjögren’s syndrome as a systemic disease: a study of participants enrolled in an international Sjögren’s syndrome registry. Arthritis Care Res. 2012. doi:10.1002/acr.21610.
Jara LJ, Navarro C, Brito-Zerón Mdel P, García-Carrasco M, Escárcega RO, Ramos-Casals M. Thyroid disease in Sjögren’s syndrome. Clin Rheumatol. 2007;26:1601–6.
Lafitte C, Amoura Z, Cacoub P, Pradat-Diehl P, Picq C, Salachas F, et al. Neurological complications of primary Sjögren’s syndrome. J Neurol. 2001;248:577–84.
Harboe E, Tjensvoll AB, Maroni S, Gøransson LG, Greve OJ, Beyer MK, et al. Neuropsychiatric syndromes in patients with systemic lupus erythematosus and primary Sjögren syndrome: a comparative population-based study. Ann Rheum Dis. 2009;68:1541–6.
Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM. Hematologic manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspect. Medicine. 2009;8:284–93.
Tsokos M, Lazarou SA, Moutsopoulos HM. Vasculitis in primary Sjögren’s syndrome. Histologic classification and clinical presentation. Am J Clin Pathol. 1987;88:26–31.
Goëb V, Salle V, Duhaut P, Jouen F, Smail A, Ducroix JP, et al. Clinical significance of autoantibodies recognizing Sjögren’s syndrome A (SSA), SSB, calpastatin and alpha-fodrin in primary Sjögren’s syndrome. Clin Exp Immunol. 2007;148:281–7.
Sène D, Jallouli M, Lefaucheur JP, Saadoun D, Costedoat-Chalumeau N, Maisonobe T, et al. Peripheral neuropathies associated with primary Sjögren syndrome: immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy. Medicine. 2011;90:133–8.
Molina R, Provost TT, Alexander EL. Peripheral inflammatory vascular disease in Sjögren’s syndrome. Association with nervous system complications. Arthritis Rheum. 1985;28:1341–7.
Ramos-Casals M, Anaya JM, García-Carrasco M, Rosas J, Bové A, Claver G, et al. Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients. Medicine. 2004;83:96–106.
Ramos-Casals M, Brito-Zerón P, Yagüe J, Akasbi M, Bautista R, Ruano M, et al. Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren’s syndrome. Rheumatology. 2005;44:89–94.
Pezzi PP, Paroli MP, Priori R, Da Dalt S, Corradi R. Vogt-Koyanagi-Harada syndrome and keratoconjunctivitis sicca. Am J Ophthalmol. 2004;137:769–70.
Ebbo M, Daniel L, Pavic M, Sève P, Hamidou M, Andres E, et al. IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry. Medicine. 2012;91:49–56.
Vitali C, Bombardieri S, Moutsopoulos HM, Balestrieri G, Bencivelli W, Bernstein RM, et al. Preliminary criteria for the classification of Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthitis Rheum. 1993;36:340–7.
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61:554–8.
Danjo Y. Diagnostic usefulness and cutoff value of Schirmer’s I test in the Japanese diagnostic criteria of dry eye. Graefes Arch Clin Exp Ophthalmol. 1997;235:761–6.
Caffery B, Simpson T, Wang S, Bailey D, McComb J, Rutka J, et al. Rose bengal staining of the temporal conjunctiva differentiates Sjögren’s syndrome from keratoconjunctivitis sicca. Invest Ophthalmol Vis Sci. 2010;51:2381–7.
Korb DR, Herman JP, Finnemore VM, Exford JM, Blackie CA. An evaluation of the efficacy of fluorescein, rose bengal, lissamine green, and a new dye mixture for ocular surface staining. Eye Contact Lens. 2008;34:61–4.
Whitcher JP, Shiboski CH, Shiboski SC, Heidenreich AM, Kitagawa K, Zhang S, et al. A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren’s Syndrome International Registry. Am J Ophthalmol. 2010;149:405–15.
Obinata K, Sato T, Ohmori K, Shindo M, Nakamura M. A comparison of diagnostic tools for Sjögren syndrome, with emphasis on sialography, histopathology, and ultrasonography. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010;109:129–34.
Vivino FB, Hermann GA. Role of nuclear scintigraphy in the characterization and management of the salivary component of Sjögren’s syndrome. Rheum Dis Clin North Am. 2008;34:973–86.
Daniels TE, Silverman Jr S, Michalski JP, Greenspan JS, Sylvester RA, Talal N. The oral component of Sjögren’s syndrome. Oral Surg Oral Med Oral Pathol. 1975;39:875–85.
Stewart CM, Bhattacharyya I, Berg K, Cohen DM, Orlando C, Drew P, et al. Labial salivary gland biopsies in Sjögren’s syndrome: still the gold standard? Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008;106:392–402.
De Wilde PC, Baak JP, van Houwelingen JC, Kater L, Slootweg PJ. Morphometric study of histological changes in sublabial salivary glands due to aging process. J Clin Pathol. 1986;39:406–17.
Takeda Y, Komori A. Focal lymphocytic infiltration in the human labial salivary glands: a postmortem study. J Oral Pathol. 1986;15:83–6.
Radfar L, Kleiner DE, Fox PC, Pillemer SR. Prevalence and clinical significance of lymphocytic foci in minor salivary glands of healthy volunteers. Arthritis Rheum. 2002;47:520–4.
Lindahl G, Hedfors E. Focal lymphocytic infiltrates of salivary glands are not confined to Sjögren’s syndrome. Scand J Rheumatol Suppl. 1986;61:52–5.
Hauschild S, Schmitt WH, Csernok E, Flesch BK, Rautmann A, Gross WL. ANCA in systemic vasculitides, collagen vascular diseases, rheumatic disorders and inflammatory bowel diseases. Adv Exp Med Biol. 1993;336:245–51.
Nishiya K, Chikazawa H, Hashimoto K, Miyawaki S. Antineutrophil cytoplasmic antibody in patients with primary Sjögren’s syndrome. Clin Rheumatol. 1999;18:268–71.
Ramos-Casals M, Nardi N, Brito-Zerón P, Aguiló S, Gil V, Delgado G, et al. Atypical autoantibodies in patients with primary Sjögren syndrome: clinical characteristics and follow-up of 82 cases. Semin Arthritis Rheum. 2006;35:312–21.
Lin DF, Yan SM, Zhao Y, Zhang W, Li MT, Zeng XF, et al. Clinical and prognostic characteristics of 573 cases of primary Sjögren’s syndrome. Chin Med J (Engl). 2010;123:3252–7.
Gottenberg JE, Mignot S, Nicaise-Rolland P, Cohen-Solal J, Aucouturier F, Goetz J, et al. Prevalence of anti-cyclic citrullinated peptide and anti-keratin antibodies in patients with primary Sjögren’s syndrome. Ann Rheum Dis. 2005;64:114–7.
Ulbricht KU, Schmidt RE, Witte T. Antibodies against alpha-fodrin in Sjögren’s syndrome. Autoimmun Rev. 2003;2:109–13.
Huang QS, LE AP, Luo ZQ, Li JM, Wang WQ, Ju BH, et al. Diagnostic value of anti-alpha-fodrin antibody testing for Sjögren’s syndrome: a meta-analysis [Article in Chinese]. Zhonghua Yi Xue Za Zhi. 2008;88:2971–6.
Vivino FB, Al-Hashimi I, Khan Z, LeVeque FG, Salisbury 3rd PL, Tran-Johnson TK, et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed-dose, multicenter trial. Arch Intern Med. 1999;159:174–81.
Petrone D, Condemi JJ, Fife R, Gluck O, Cohen S, Dalgin P. A double-blind, randomized, placebo-controlled study of cevimeline in Sjögren’s syndrome patients with xerostomia and keratoconjunctivitis sicca. Arthritis Rheum. 2002;46:748–54.
Sall K, Stevenson OD, Mundorf TK, Reis BL. Two multicenter, randomized studies of the efficacy and safety of cyclosporine ophthalmic emulsion in moderate to severe dry eye disease. Ophthalmology. 2000;107:631–9.
Stevenson D, Tauber J, Reis BL. Efficacy and safety of cyclosporin A ophthalmic emulsion in the treatment of moderate-to-severe dry eye disease: a dose-ranging, randomized trial. Ophthalmology. 2000;107:967–74.
Kruize AA, Hené RJ, Kallenberg CG, van Bijsterveld OP, van der Heide A, Kater L, et al. Hydroxychloroquine treatment for primary Sjögren’s syndrome: a two year double blind crossover trial. Ann Rheum Dis. 1993;52:360–4.
Mariette X, Ravaud P, Steinfeld S, Baron G, Goetz J, Hachulla E, et al. Inefficacy of infliximab in primary Sjögren’s syndrome: results of the randomized, controlled Trial of Remicade in Primary Sjögren’s Syndrome (TRIPSS). Arthritis Rheum. 2004;50:1270–6.
Sankar V, Brennan MT, Kok MR, Leakan RA, Smith JA, Manny J, et al. Etanercept in Sjögren’s syndrome: a twelve-week randomized, double-blind, placebo-controlled pilot clinical trial. Arthritis Rheum. 2004;50:2240–5.
Meijer JM, Meiners PM, Vissink A, Spijkervet FK, Abdulahad W, Kamminga N, et al. Effectiveness of rituximab treatment in primary Sjögren’s syndrome: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2010;62:960–8.
Haldorsen K, Moen K, Jacobsen H, Jonsson R, Brun JG. Exocrine function in primary Sjögren syndrome: natural course and prognostic factors. Ann Rheum Dis. 2008;67:949–54.
Voulgarelis M, Tzioufas AG, Moutsopoulos HM. Mortality in Sjögren’s syndrome. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S66–71.
Hietaharju A, Yli-Kerttula U, Häkkinen V, Frey H. Nervous system manifestations in Sjögren’s syndrome. Acta Neurol Scand. 1990;81:144–52.
Moll JW, Markusse HM, Pijnenburg JJ, Vecht CJ, Henzen-Logmans SC. Antineuronal antibodies in patients with neurologic complications of primary Sjögren’s syndrome. Neurology. 1993;43:2574–81.
Gemignani F, Marbini A, Pavesi G, Di Vittorio S, Manganelli P, Cenacchi G, et al. Peripheral neuropathy associated with primary Sjögren’s syndrome. J Neurol Neurosurg Psychiatry. 1994;57:983–6.
Govoni M, Bajocchi G, Rizzo N, Tola MR, Caniatti L, Tugnoli V, et al. Neurological involvement in primary Sjögren’s syndrome: clinical and instrumental evaluation in a cohort of Italian patients. Clin Rheumatol. 1999;18:299–303.
Barendregt PJ, van den Bent MJ, van Raaij-van den Aarssen VJ, van den Meiracker AH, Vecht CJ, van der Heijde GL, et al. Involvement of the peripheral nervous system in primary Sjögren’s syndrome. Ann Rheum Dis. 2001;60:876–81.
Gøransson LG, Herigstad A, Tjensvoll AB, Harboe E, Mellgren SI, Omdal R. Peripheral neuropathy in primary sjogren syndrome: a population-based study. Arch Neurol. 2006;63:1612–5.
Mellgren SI, Conn DL, Stevens JC, Dyck PJ. Peripheral neuropathy in primary Sjögren’s syndrome. Neurology. 1989;39:390–4.
Grant IA, Hunder GG, Homburger HA, Dyck PJ. Peripheral neuropathy associated with sicca complex. Neurology. 1997;48:855–62.
Miwa Y, Inokuma S, Yokue Y, Okazaki Y, Sato T, Maezawa R. Peripheral nervous system involvement complicated due to vasculitis in cases of primary Sjögren’s syndrome: case studies in a single hospital and a literature review. Mod Rheumatol. 2003;13:160–7.
Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine. 2004;83:280–91.
Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, et al. The wide spectrum of clinical manifestations in Sjögren’s syndrome-associated neuropathy. Brain. 2005;128:2518–34.
Vrethem M, Lindvall B, Holmgren H, Henriksson KG, Lindström F, Ernerudh J. Neuropathy and myopathy in primary Sjögren’s syndrome: neurophysiological, immunological and muscle biopsy results. Acta Neurol Scand. 1990;82:126–31.
Kaltreider HB, Talal N. The neuropathy of Sjögren’s syndrome. Trigeminal nerve involvement. Ann Intern Med. 1969;70:751–62.
Selva O’Callaghan A, Bosch Gil JA, Solans Laque R, Segura García A, Armadans Gil L, Mijares Boeckh-Behrens T, et al. Sindrome de Sjogren primario: caracteristicas clinicas e inmunologicas de 114 pacientes. Med Clin (Barc). 2001;116:721–75.
Chai J, Herrmann DN, Stanton M, Barbano RL, Logigian EL. Painful small-fiber neuropathy in Sjogren syndrome. Neurology. 2005;65:925–7.
ter Borg EJ, Risselada AP, Kelder JC. Relation of systemic autoantibodies to the number of extraglandular manifestations in primary Sjögren’s syndrome: a retrospective analysis of 65 patients in the Netherlands. Semin Arthritis Rheum. 2011;40:547–51.
Andonopoulos AP, Lagos G, Drosos AA, Moutsopoulos HM. The spectrum of neurological involvement in Sjogren’s syndrome. Br J Rheumatol. 1990;29:21–3.
Font J, Ramos-Casals M, de la Red G, Pou A, Casanova A, García-Carrasco M, et al. Pure sensory neuropathy in primary Sjögren’s syndrome. Longterm prospective followup and review of the literature. J Rheumatol. 2003;30:1552–7.
Font J, Valls J, Cervera R, Pou A, Ingelmo M, Graus F. Pure sensory neuropathy in patients with primary Sjögren’s syndrome: clinical, immunological, and electromyographic findings. Ann Rheum Dis. 1990;49:775–8.
Denislic M, Meh D. Early asymmetric neuropathy in primary Sjögren’s syndrome. J Neurol. 1997;244:383–7.
Takahashi Y, Takata T, Hoshino M, Sakurai M, Kanazawa I. Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren’s syndrome. IV immunoglobulin. Neurology. 2003;60:503–5.
Kaplan JG, Rosenberg R, Reinitz E, Buchbinder S, Schaumburg HH. Invited review: peripheral neuropathy in Sjogren’s syndrome. Muscle Nerve. 1990;13:570–9.
Auger RG, Windebank AJ, Lucchinetti CF, Chalk CH. Role of the blink reflex in the evaluation of sensory neuronopathy. Neurology. 1999;53:407–8.
Valls-Sole J, Graus F, Font J, Pou A, Tolosa ES. Normal proprioceptive trigeminal afferents in patients with Sjögren’s syndrome and sensory neuronopathy. Ann Neurol. 1990;28:786–90.
Lopate G, Pestronk A, Al-Lozi M, Lynch T, Florence J, Miller T, et al. Peripheral neuropathy in an outpatient cohort of patients with Sjögren’s syndrome. Muscle Nerve. 2006;33:672–6.
Pavlakis PP, Alexopoulos H, Kosmidis ML, Stamboulis E, Routsias JG, Tzartos SJ, et al. Peripheral neuropathies in Sjogren syndrome: a new reappraisal. J Neurol Neurosurg Psychiatry. 2011;82:798–802.
Denislic M, Meh D. Neurophysiological assessment of peripheral neuropathy in primary Sjögren’s syndrome. Clin Investig. 1994;72:822–9.
Fauchais AL, Richard L, Gondran G, Ghorab K, Palat S, Bezanahary H, et al. Neuropathie des petites fibres au cours du syndrome de Sjogren primitive. Rev Med Interne. 2011;32:142–8.
Gross M. Chronic relapsing inflammatory polyneuropathy complicating sicca syndrome. J Neurol Neurosurg Psychiatry. 1987;5:939–40.
Chen WH, Yeh JH, Chiu HC. Plasmapheresis in the treatment of ataxic sensory neuropathy associated with Sjögren’s syndrome. Eur Neurol. 2001;45:270–4.
Rigamonti A, Lauria G, Balgera R, Agostoni E. Subacute inflammatory polyradiculopathy associated with Sjögren’s syndrome. Muscle Nerve. 2009;39:855–7.
Katz JS, Houroupian D, Ross MA. Multisystem neuronal involvement and sicca complex: broadening the spectrum of complications. Muscle Nerve. 1999;22:404–7.
Hagiwara K, Murai H, Ochi H, Osoegawa M, Shigeto H, Ohyagi Y, et al. Upper motor neuron syndrome associated with subclinical Sjögren’s syndrome. Intern Med. 2008;47:1047–51.
Rafai MA, Boulaajaj FZ, Moutawakil F, Addali N, El Moutawakkil B, Fadel H, et al. Neurological manifestations revealing primitive Gougerot-Sjogren syndrome: 9 cases. Joint Bone Spine. 2009;76:139–45.
Takakura Y, Murai H, Furuya H, Ochi H, Kira J. A case of brachial amyotrophic diplegia accompanied with Sjögren’s syndrome presenting good response to immunotherapies in the early course of the disease [Japanese]. Rinsho Shinkeigaku. 2005;45:346–50.
Molina R, Provost TT, Arnett FC, Bias WB, Hochberg MC, Wilson RW, et al. Primary Sjögren’s syndrome in men. Clinical, serologic, and immunogenetic features. Am J Med. 1986;80:23–31.
Mauch E, Völk C, Kratzsch G, Krapf H, Kornhuber HH, Laufen H, et al. Neurological and neuropsychiatric dysfunction in primary Sjögren’s syndrome. Acta Neurol Scand. 1994;89:31–5.
Friedman JA, Miller EB, Green L, Huszar M, Schattner A. A community-based cohort of 201 consecutive patients with primary Sjögren’s syndrome in Israel: Ashkenazi patients compared with those of Sephardic descent. Clin Exp Rheumatol. 2006;23:274–80.
Tajima Y, Mito Y, Owada Y, Tsukishima E, Moriwaka F, Tashiro K. Neurological manifestations of primary Sjögren’s syndrome in Japanese patients. Intern Med. 1997;36:690–3.
Pou Serradell A, Gaya JV. Trois cas de neuropathies peripheriques rares associees au syndrome de Gougerot-Sjogren primitif. Rev Neurol (Paris). 1993;149:481–4.
Mekinian A, Ravaud P, Hatron PY, Larroche C, Leone J, Gombert B, et al. Efficacy of rituximab in primary Sjogren’s syndrome with peripheral nervous system involvement: results from the AIR registry. Ann Rheum Dis. 2012;71:84–7.
Ashraf VV, Bhasi R, Kumar RP, Girija AS. Primary Sjögren’s syndrome manifesting as multiple cranial neuropathies: MRI findings. Ann Indian Acad Neurol. 2009;12:124–6.
Vincent D, Loron P, Awada A, Gautier JC. Paralysies multiples et recidivantes des nerfs craniens. Syndrome de Gougerot-Sjogren. Rev Neurol (Paris). 1985;141:318–21.
Touzé E, Blanche P, Zuber M. A 35-year history of recurrent multiple cranial neuropathy due to primary Sjögren’s syndrome. J Neurol. 1999;246:968–9.
Rist S, Sellam J, Hachulla E, Sordet C, Puéchal X, Hatron PY, et al. Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren’s syndrome: a national multicentric retrospective study. Arthritis Care Res. 2011;63:1339–44.
Asahina M, Kuwabara S, Asahina M, Nakajima M, Hattori T. D-penicillamine treatment for chronic sensory ataxic neuropathy associated with Sjögren’s syndrome. Neurology. 1998;51:1451–3.
Yamada S, Mori K, Matsuo K, Inukai A, Kawagashira Y, Sobue G. Interferon alfa treatment for Sjogren’s syndrome associated neuropathy. J Neurol Neurosurg Psychiatry. 2005;76:576–8.
Caroyer JM, Manto MU, Steinfeld SD. Severe sensory neuronopathy responsive to infliximab in primary Sjögren’s syndrome. Neurology. 2002;59:1113–4.
Gorson KC, Natarajan N, Ropper AH, Weinstein R. Rituximab treatment in patients with IVIg-dependent immune polyneuropathy: a prospective pilot trial. Muscle Nerve. 2007;35:66–9.
Morozumi S, Kawagashira Y, Iijima M, Koike H, Hattori N, Katsuno M, et al. Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjögren’s syndrome. J Neurol Sci. 2009;279:57–61.
Wakasugi D, Kato T, Gono T, Ito E, Nodera H, Kawaguchi Y, et al. Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren’s syndrome. Mod Rheumatol. 2009;19:437–40.
Tassiulis IO, Boumpas DT. Clinical features and treatment of systemic lupus erythematosus. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1263–300.
Hahn BH. Chapter 319: Systemic lupus erythematosus. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison’s principles of internal medicine. 18th edn. New York: McGraw-Hill; 2012. http://www.accessmedicine.com/content.aspx?aID=9136499. Accessed 27 Dec 2011.
Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365:2110–21.
Pons-Estel GJ, Alarcón GS, Scofield L, Reinlib L, Cooper GS. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum. 2010;39:257–68.
Domsic RT, Ramsey-Golden R, Manzi S. Epidemiology and classification of systemic lupus erythematosus. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, editors. Rheumatology. 4th ed. Philadelphia: Mosby/Elsevier; 2007. p. 1211–6.
Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med. 2008;358:929–39.
Hahn BH, Tsao BP. Pathogenesis of systemic lupus erythematosus. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1233–62.
Crispín JC, Liossis SN, Kis-Toth K, Lieberman LA, Kyttaris VC, Juang YT, et al. Pathogenesis of human systemic lupus erythematosus: recent advances. Trends Mol Med. 2010;16:47–57.
Muñoz LE, Janko C, Schulze C, Schorn C, Sarter K, Schett G, et al. Autoimmunity and chronic inflammation – two clearance-related steps in the etiopathogenesis of SLE. Autoimmun Rev. 2010;10:38–42.
Fransen JH, van der Vlag J, Ruben J, Adema GJ, Berden JH, Hilbrands LB. The role of dendritic cells in the pathogenesis of systemic lupus erythematosus. Arthritis Res Ther. 2010;12:207.
Rönnblom L, Alm GV, Eloranta ML. The type I interferon system in the development of lupus. Semin Immunol. 2011;23:113–21.
Perry D, Peck AB, Carcamo WC, Morel L, Nguyen CQ. The current concept of T (h) 17 cells and their expanding role in systemic lupus erythematosus. Arthritis. 2011;2011:810649.
Kaiser R, Criswell LA. Genetics research in systemic lupus erythematosus for clinicians: methodology, progress, and controversies. Curr Opin Rheumatol. 2010;22:119–25.
Sestak AL, Fürnrohr BG, Harley JB, Merrill JT, Namjou B. The genetics of systemic lupus erythematosus and implications for targeted therapy. Ann Rheum Dis. 2011;70 Suppl 1Suppl 1:i37–43.
Peterson KS, Winchester RJ. Systemic lupus erythematosus: pathogenesis. In: Koopman WJ, Moreland LW, editors. Arthritis and allied conditions. 15th ed. Philadelphia: Lippincott, Williams & Wilkins; 2005.
Hoffmann MH, Trembleau S, Muller S, Steiner G. Nucleic acid-associated autoantigens: pathogenic involvement and therapeutic potential. J Autoimmun. 2010;34:J178–206.
Petri MA. Systemic lupus erythematosus: clinical aspects. In: Koopman WJ, Moreland LW, editors. Arthritis and allied conditions. 15th ed. Philadelphia: Lippincott, Williams & Wilkins; 2005.
Gladman DD, Urowitz MB. Clinical features of systemic lupus erythematosus. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, editors. Rheumatology. 4th ed. Philadelphia: Mosby/Elsevier; 2007. p. 1277–97.
Ruperto N, Hanrahan LM, Alarcón GS, Belmont HM, Brey RL, Brunetta P, et al. International consensus for a definition of disease flare in lupus. Lupus. 2011;20:453–62.
Saxena R, Mahajan T, Mohan C. Lupus nephritis: current update. Arthritis Res Ther. 2011;13:240.
Ad ACR. Hoc Committee on Neuropsychiatric Lupus Nomenclature. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999;42:599–608.
Mitsikostas DD, Sfikakis PP, Goadsby PJ. A meta-analysis for headache in systemic lupus erythematosus: the evidence and the myth. Brain. 2004;127:1200–9.
Ainiala H, Loukkola J, Peltola J, Korpela M, Hietaharju A. The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus. Neurology. 2001;57:496–500.
Brey RL, Holliday SL, Saklad AR, Navarrete MG, Hermosillo-Romo D, Stallworth CL, et al. Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology. 2002;58:1214–20.
Drenkard C, Villa AR, Reyes E, Abello M, Alarcón-Segovia D. Vasculitis in systemic lupus erythematosus. Lupus. 1997;6:235–42.
Ramos-Casals M, Nardi N, Lagrutta M, Brito-Zerón P, Bové A, Delgado G, et al. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine. 2006;85:95–104.
Bertoli AM, Vilá LM, Alarcón GS, McGwin G, Edberg JC, Petri M, et al. Factors associated with arterial vascular events in PROFILE: a Multiethnic Lupus Cohort. Lupus. 2009;18:958–65.
Makol A, Petri M. Pancreatitis in systemic lupus erythematosus: frequency and associated factors - a review of the Hopkins Lupus Cohort. J Rheumatol. 2010;37:341–5.
Feinglass EJ, Arnett FC, Dorsch CA, Zizic TM, Stevens MB. Neuropsychiatric manifestations of systemic lupus erythematosus: diagnosis, clinical spectrum, and relationship to other features of the disease. Medicine. 1976;55:323–39.
Hochberg MC, Boyd RE, Ahearn JM, Arnett FC, Bias WB, Provost TT, et al. Systemic lupus erythematosus: a review of clinico-laboratory features and immunogenetic markers in 150 patients with emphasis on demographic subsets. Medicine. 1985;64:285–95.
Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271–7.
Mosca M, Tani C, Aringer M, Bombardieri S, Boumpas D, Brey R, et al. European League Against Rheumatism recommendations for monitoring patients with systemic lupus erythematosus in clinical practice and in observational studies. Ann Rheum Dis. 2010;69:1269–74.
Tektonidou MG, Laskari K, Panagiotakos DB, Moutsopoulos HM. Risk factors for thrombosis and primary thrombosis prevention in patients with systemic lupus erythematosus with or without antiphospholipid antibodies. Arthritis Rheum. 2009;61:29–36.
Firooz N, Albert DA, Wallace DJ, Ishimori M, Berel D, Weisman MH. High-sensitivity C-reactive protein and erythrocyte sedimentation rate in systemic lupus erythematosus. Lupus. 2011;20:588–97.
Ruiz-Irastorza G, Ramos-Casals M, Brito-Zeron P, Khamashta MA. Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: a systematic review. Ann Rheum Dis. 2010;69:20–8.
Hepburn AL, Narat S, Mason JC. The management of peripheral blood cytopenias in systemic lupus erythematosus. Rheumatology. 2010;49:2243–54.
Lee YH, Woo JH, Choi SJ, Ji JD, Song GG. Induction and maintenance therapy for lupus nephritis: a systematic review and meta-analysis. Lupus. 2010;19:703–10.
Touma Z, Gladman DD, Urowitz MB, Beyene J, Uleryk EM, Shah PS. Mycophenolate mofetil for induction treatment of lupus nephritis: a systematic review and metaanalysis. J Rheumatol. 2011;38:69–78.
Chen W, Tang X, Liu Q, Chen W, Fu P, Liu F, et al. Short-term outcomes of induction therapy with tacrolimus versus cyclophosphamide for active lupus nephritis: a multicenter randomized clinical trial. Am J Kidney Dis. 2011;57:235–44.
Merrill JT, Neuwelt CM, Wallace DJ, Shanahan JC, Latinis KM, Oates JC, et al. Efficacy and safety of rituximab in moderately-to-severely active systemic lupus erythematosus: the randomized, double-blind, phase II/III systemic lupus erythematosus evaluation of rituximab trial. Arthritis Rheum. 2010;62:222–33.
Rovin BH, Furie R, Latinis K, Looney RJ, Fervenza FC, Sanchez-Guerrero J, et al. Efficacy and safety of rituximab in patients with active proliferative lupus nephritis: the lupus nephritis assessment with rituximab (LUNAR) study. Arthritis Rheum. 2012. doi:10.1002/art.34359.
Burness CB, McCormack PL. Belimumab: in systemic lupus erythematosus. Drugs. 2011;71:2435–44.
Navarra SV, Guzmán RM, Gallacher AE, Hall S, Levy RA, Jimenez RE, et al. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet. 2011;377:721–31.
Furie R, Petri M, Zamani O, Cervera R, Wallace DJ, Tegzová D, et al. A phase III, randomized, placebo-controlled study of belimumab, a monoclonal antibody that inhibits B lymphocyte stimulator, in patients with systemic lupus erythematosus. Arthritis Rheum. 2011;63:3918–30.
Ippolito A, Petri M. An update on mortality in systemic lupus erythematosus. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S72–9.
Bernatsky S, Boivin JF, Joseph L, Rajan R, Zoma A, Manzi S, et al. An international cohort study of cancer in systemic lupus erythematosus. Arthritis Rheum. 2005;52:1481–90.
Drenkard C, Villa AR, Garcia-Padilla C, Pérez-Vázquez ME, Alarcón-Segovia D. Remission of systematic lupus erythematosus. Medicine. 1996;75:88–98.
Khedr EM, Khedr T, Farweez HM, Abdella G, El Beih EA. Multimodal electroneurophysiological studies of systemic lupus erythematosus. Neuropsychobiology. 2001;43:204–12.
Gøransson LG, Tjensvoll AB, Herigstad A, Mellgren SI, Omdal R. Small-diameter nerve fiber neuropathy in systemic lupus erythematosus. Arch Neurol. 2006;63:401–4.
Tseng MT, Hsieh SC, Shun CT, Lee KL, Pan CL, Lin WM, et al. Skin denervation and cutaneous vasculitis in systemic lupus erythematosus. Brain. 2006;129:977–85.
Servioli L, Pérez C, Consani S, Suárez A, Sehabiaga G, Collazo C, et al. Prevalence and characteristics of immunomediated neuropathies in a group of patients with autoimmune diseases. J Clin Neuromuscul Dis. 2007;9:285–90.
Honczarenko K, Budzianowska A, Ostanek L. Neurological syndromes in systemic lupus erythematosus and their association with antiphospholipid syndrome. Neurol Neurochir Pol. 2008;42:513–7.
Omdal R, Henriksen OA, Mellgren SI, Husby G. Peripheral neuropathy in systemic lupus erythematosus. Neurology. 1991;41:808–11.
McNicholl JM, Glynn D, Mongey AB, Hutchinson M, Bresnihan B. A prospective study of neurophysiologic, neurologic and immunologic abnormalities in systemic lupus erythematosus. J Rheumatol. 1994;21:1061–6.
Laversuch CJ, Seo H, Modarres H, Collins DA, McKenna W, Bourke BE. Reduction in heart rate variability in patients with systemic lupus erythematosus. J Rheumatol. 1997;24:1540–4.
Fierro B, Brighina F, Amico L, Aloisio A, Buffa D, Caravaglios G, et al. Evoked potential study and radiological findings in patients with systemic lupus erythematosus. Electromyogr Clin Neurophysiol. 1999;39:305–13.
Huynh C, Ho S-L, Fong K-Y, Cheung RT, Mok C-C, Lau C-S. Peripheral neuropathy in systemic lupus erythematosus. J Clin Neurophysiol. 1999;16:164–8.
Campello I, Almárcegui C, Velilla J, Hortells JL, Oliveros A. Neuropatia periferica en el lupus eritematoso sistemico. Rev Neurol. 2001;33:27–30.
Martinez-Taboada VM, Alonso RB, Armona J, Fernandez-Sueiro JL, Vela CG, Rogriguez-Valverde V. Mononeuritis multiplex in systemic lupus erythematosus: response to pulse intravenous cyclophosphamide. Lupus. 1996;5:74–6.
Hughes RA, Cameron JS, Hall SM, Heaton J, Payan J, Teoh R. Multiple mononeuropathy as the initial presentation of systemic lupus erythematosus – nerve biopsy and response to plasma exchange. J Neurol. 1982;228:239–47.
McCombe PA, McLead JG, Pollard JD, Guo YP, Ingall TJ. Peripheral sensorimotor and autonomic neuropathy associated with systemic lupus erythematosus. Clinical, pathological, and immunological features. Brain. 1987;110:533–49.
Markusse HM, Vroom TM, Heurkens AH, Vecht CJ. Polyneuropathy as initial manifestation of necrotizing vasculitis and gangrene in systemic lupus erythematosus. Neth J Med. 1991;38:204–8.
Bodi I, Varadi P, Pokorny G, Engelhardt J, Dibó G, Vécsei L, et al. Polyneuropathy with endoneurial immune complex deposition as the first manifestation of systemic lupus erythematosus. Acta Neuropathol. 1998;96:297–300.
Enevoldson TP, Wiles CM. Severe vasculitic neuropathy in systemic lupus erythematosus and response to cyclophosphamide. J Neurol Neurosurg Psychiatry. 1991;54:468.
Stefurak T, Midroni G, Bilbao J. Vasculitic polyradiculopathy in systemic lupus erythematosus. J Neurol Neurosurg Psychiatry. 1999;66:658–61.
Honda M. Neurological aspect of systemic lupus erythematosus. Keio J Med. 1966;15:139–64.
Johnson RT, Richardson EP. The neurological manifestations of systemic lupus erythematosus: a clinical-pathological study of 24 cases and review of the literature. Medicine. 1968;47:337–69.
Omdal R, Løseth S, Torbergsen T, Koldingsnes W, Husby G, Mellgren SI. Peripheral neuropathy in systemic lupus erythematosus – a longitudinal study. Acta Neurol Scand. 2001;103:386–91.
Scheinberg L. Polyneuritis in systemic lupus erythematosus: review of the literature and report of a case. N Engl J Med. 1956;255:416–21.
Rechthand E, Cornblath DR, Stern BJ, Meyerhoff JO. Chronic demyelinating polyneuropathy in systemic lupus erythematosus. Neurology. 1984;34:1375–7.
Sindern S, Stark E, Haas J, Steck AJ. Serum antibodies to GM1 and GM3-gangliosides in systemic lupus erythematosus with chronic inflammatory demyelinating polyradiculoneuropathy. Acta Neurol Scand. 1991;83:309–402.
Lesprit P, Mouloud F, Bierling P, Schaeffer A, Cesaro P, Brun-Buisson C, et al. Prolonged remission of SLE-associated polyradiculoneuropathy after a single course of intravenous immunoglobulin. Scand J Rheumatol. 1996;25:177–9.
Hantson P, Kevers L, Fabien N, Van Den Bergh P. Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement, dysautonomia, respiratory failure, and autoantibodies. Muscle Nerve. 2010;41:423–6.
Zoilo MA, Eduardo B, Enrique F, del Rocio MV. Chronic inflammatory demyelinating polyradiculoneuropathy in a boy with systemic lupus erythematosus. Rheumatol Int. 2010;30:965–8.
Jabs DA, Miller NR, Newman SA, Johnson MA, Stevens MB. Optic neuropathy in systemic lupus erythematosus. Arch Ophthalmol. 1986;104:564–8.
Lin YC, Wang AG, Yen MY. Systemic lupus erythematosus-associated optic neuritis: clinical experience and literature review. Acta Ophthalmol. 2009;87:204–10.
Frigui M, Frikha F, Sellemi D, Chouayakh F, Feki J, Bahloul Z. Optic neuropathy as a presenting feature of systemic lupus erythematosus: two case reports and literature review. Lupus. 2011;20:1214–8.
Karatas E, Onat AM, Durucu C, Baglam T, Kanlikama M, Altunoren O, et al. Audiovestibular disturbance in patients with systemic lupus erythematosus. Otolaryngol Head Neck Surg. 2007;136:82–6.
Gomides AP, do Rosário EJ, Borges HM, Gomides HH, de Pádua PM, Sampaio-Barros PD. Sensorineural dysacusis in patients with systemic lupus erythematosus. Lupus. 2007;16:987–90.
Lundberg PO, Werner I. Trigeminal neuropathy in systemic lupus erythematosus. Acta Neurol Scand. 1972;48:330–40.
Rosenstein ED, Sobelman J, Kramer N. Isolated, pupil-sparing third nerve palsy as initial manifestation of systemic lupus erythematosus. J Clin Neuroophthalmol. 1989;9:285–8.
Blaustein DA, Blaustein SA. Antinuclear antibody negative systemic lupus erythematosus presenting as bilateral facial paralysis. J Rheumatol. 1998;25:798–800.
Khalidi NA, Rebello R, Robertson DD. Sensorineural hearing loss in systemic lupus erythematosus: case report and literature review. J Laryngol Otol. 2008;122:1371–6.
Hughes M, Hill J. Left vocal cord paralysis in systemic lupus erythematosus. Mod Rheumatol. 2009;19:441–2.
Saleh Z, Menassa J, Abbas O, Atweh S, Arayssi T. Cranial nerve VI palsy as a rare initial presentation of systemic lupus erythematosus: case report and review of the literature. Lupus. 2010;19:201–5.
Figueroa JJ, Tabechian D, Herrmann DN. Severe long-standing dysimmune sensory neuronopathy responsive to etanercept. J Clin Neuromuscul Dis. 2008;9:415–20.
Wang JC, Lin YC, Yang TF, Lin HY. Ataxic sensory neuronopathy in a patient with systemic lupus erythematosus. Lupus. 2012. doi:10.1177/0961203311434105.
Magnani G, Nemni R, Leocani S, Amadio S, Locatelli T, Maderna L, et al. Neuromyotonia, systemic lupus erythematosus and acetylcholine-receptor antibodies. J Neurol. 1998;245:182–5.
Hardy K, Herry I, Attali V, Cadranel J, Similowski T. Bilateral phrenic paralysis in a patient with systemic lupus erythematosus. Chest. 2001;119:1274–7.
Maldonado ME, Williams Jr RC, Adair JC, Hart BL, Gregg L, Sibbitt Jr WL. Neuropsychiatric systemic lupus erythematosus presenting as amyotrophic lateral sclerosis. J Rheumatol. 2002;29:633–5.
Barile-Fabris L, Ariza-Andraca R, Olguín-Ortega L, Jara LJ, Fraga-Mouret A, Miranda-Limón JM, et al. Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann Rheum Dis. 2005;64:620–5.
Varga J, Denton CP. Systemic sclerosis and the scleroderma-spectrum disorders. In: Firestein GS, Budd RC, Harris ED, McInnis IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Saunders/Elsevier; 2009. p. 1311–51.
Varga J. Chapter 323: Systemic sclerosis (scleroderma) and related disorders. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison’s principles of internal medicine. 18th edn. New York: McGraw-Hill; 2012. http://www.accessmedicine.com/content.aspx?aID=9137441. Accessed 27 Dec 2011.
Silver RM, Medsger TA, Bolster MB. Systemic sclerosis and scleroderma variants: clinical aspects. In: Koopman WJ, Moreland LW, editors. Arthritis and allied conditions. 15th ed. Philadelphia: Lippincott, Williams & Wilkins; 2005.
Hachulla E, Launay D. Diagnosis and classification of systemic sclerosis. Clin Rev Allergy Immunol. 2011;40:78–83.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202–5.
Carr RD, Heisel EB, Stevenson TD. CRST syndrome. A benign variant of scleroderma. Arch Dermatol. 1965;92:519–25.
Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. 2008;37:223–35.
Ranque B, Mouthon L. Geoepidemiology of systemic sclerosis. Autoimmun Rev. 2010;9:A311–8.
Martín JE, Bossini-Castillo L, Martín J. Unraveling the genetic component of systemic sclerosis. Hum Genet. 2012. doi:10.1007/s00439-011-1137-z.
Geyer M, Müller-Ladner U. The pathogenesis of systemic sclerosis revisited. Clin Rev Allergy Immunol. 2011;40:92–103.
Katsumoto TR, Whitfield ML, Connolly MK. The pathogenesis of systemic sclerosis. Annu Rev Pathol. 2011;6:509–37.
Rabquer BJ, Koch AE. Angiogenesis and vasculopathy in systemic sclerosis: evolving concepts. Curr Rheumatol Rep. 2012;14:56–63.
Chizzolini C, Brembilla NC, Montanari E, Truchetet ME. Fibrosis and immune dysregulation in systemic sclerosis. Autoimmun Rev. 2011;10:276–81.
Gabrielli A, Svegliati S, Moroncini G, Avvedimento EV. Pathogenic autoantibodies in systemic sclerosis. Curr Opin Immunol. 2007;19:640–5.
Hamaguchi Y. Autoantibody profiles in systemic sclerosis: predictive value for clinical evaluation and prognosis. J Dermatol. 2010;37:42–53.
Varga JA, Trojanowska M. Fibrosis in systemic sclerosis. Rheum Dis Clin North Am. 2008;34:115–43.
Wei J, Bhattacharyya S, Tourtellotte WG, Varga J. Fibrosis in systemic sclerosis: emerging concepts and implications for targeted therapy. Autoimmun Rev. 2011;10:267–75.
Della Rossa A, Valentini G, Bombardieri S, Bencivelli W, Silman AJ, D’Angelo S, et al. European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres. Ann Rheum Dis. 2001;60:585–91.
Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G, et al. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine. 2002;81:139–53.
Walker UA, Tyndall A, Czirják L, Denton C, Farge-Bancel D, Kowal-Bielecka O, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis. 2007;66:754–63.
Hunzelmann N, Genth E, Krieg T, Lehmacher W, Melchers I, Meurer M, et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology. 2008;47:1185–92.
Avouac J, Walker U, Tyndall A, Kahan A, Matucci-Cerinic M, Allanore Y, et al. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database. J Rheumatol. 2010;37:1488–501.
Ebert EC. Gastric and enteric involvement in progressive systemic sclerosis. J Clin Gastroenterol. 2008;42:5–12.
Hant FN, Herpel LB, Silver RM. Pulmonary manifestations of scleroderma and mixed connective tissue disease. Clin Chest Med. 2010;31:433–49.
Penn H, Denton CP. Diagnosis, management and prevention of scleroderma renal disease. Curr Opin Rheumatol. 2008;20:692–6.
Desai CS, Lee DC, Shah SJ. Systemic sclerosis and the heart: current diagnosis and management. Curr Opin Rheumatol. 2011;23:545–54.
D’Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969;46:428–40.
Oddis CV, Eisenbeis CH, Reidbord HE, Steen VD, Medsger Jr T. Vasculitis in systemic sclerosis: association with Sjögren’s syndrome and the CREST syndrome variant. J Rheumatol. 1987;14:942–8.
Hudson M, Walker JG, Fritzler M, Taillefer S, Baron M. Hypocomplementemia in systemic sclerosis – clinical and serological correlations. J Rheumatol. 2007;34:2218–23.
Rho YH, Choi SJ, Lee YH, Ji JD, Song GG. Scleroderma associated with ANCA-associated vasculitis. Rheumatol Int. 2006;26:369–75.
Kao L, Weyand C. Vasculitis in systemic sclerosis. Int J Rheumatol. 2010;2010:385938.
Arad U, Balbir-Gurman A, Doenyas-Barak K, Amit-Vazina M, Caspi D, Elkayam O. Anti-neutrophil antibody associated vasculitis in systemic sclerosis. Semin Arthritis Rheum. 2011;41:223–9.
Endo H, Hosono T, Kondo H. Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis. J Rheumatol. 1994;21:864–70.
Casari S, Haeney M, Farrand S, Herrick A. Antineutrophil cytoplasmic antibodies a “Red Flag” in patients with systemic sclerosis. J Rheumatol. 2002;29:2666–7.
Maes B, Van Mieghem A, Messiaen T, Kuypers D, Van Damme B, Vanrenterghem Y. Limited cutaneous systemic sclerosis associated with MPO-ANCA positive renal small vessel vasculitis of the microscopic polyangiitis type. Am J Kidney Dis. 2000;36:E16.
Miyamura T, Yamamoto M, Shimada H, Suematsu E. Systemic sclerosis associated with microscopic polyangitis presenting with high myeloperoxidase (MPO) titer and necrotizing angitis: a case report [Japanese]. Ryumachi. 2002;42:910–4.
Hidalgo-Tenorio C, Càliz R, Gallego M, Jaimez L, Jiménez-Alonso J. Systemic sclerosis, pulmonary fibrosis and anti-MPO antibodies. Clin Rheumatol. 2005;24:658–60.
Wong M, Ranganath VK, Clements PJ. Antineutrophil cytoplasmic antibody-positive digital necrosis in a patient with limited systemic sclerosis. J Rheumatol. 2010;37:214–5.
Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980;23:581–90.
Reveille JD, Solomon DH. American College of Rheumatology Ad Hoc Committee of Immunologic Testing Guidelines. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum. 2003;49:399–412.
Nikpour M, Hissaria P, Byron J, Sahhar J, Micallef M, Paspaliaris W, et al. Prevalence, correlates and clinical usefulness of antibodies to RNA polymerase III in systemic sclerosis: a cross-sectional analysis of data from an Australian cohort. Arthritis Res Ther. 2011;13:R211.
Moore SC, Desantis ER. Treatment of complications associated with systemic sclerosis. Am J Health Syst Pharm. 2008;65:315–21.
Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68:620–8.
Burt RK, Milanetti F. Hematopoietic stem cell transplantation for systemic sclerosis: history and current status. Curr Opin Rheumatol. 2011;23:519–29.
Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655–66.
Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, et al. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med. 2007;176:1026–34.
Nannini C, West CP, Erwin PJ, Matteson EL. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther. 2008;10:R124.
Johnson SR, Feldman BM, Pope JE, Tomlinson GA. Shifting our thinking about uncommon disease trials: the case of methotrexate in scleroderma. J Rheumatol. 2009;36:323–9.
Herrick AL. Contemporary management of Raynaud’s phenomenon and digital ischaemic complications. Curr Opin Rheumatol. 2011;23:555–61.
Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM, Carpentier P, et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis. 2011;70:32–8.
Sitbon O, Simonneau G. Optimal management of severe pulmonary arterial hypertension. Eur Respir Rev. 2011;20:254–61.
Elhai M, Meune C, Avouac J, Kahan A, Allanore Y. Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology. 2011. doi:10.1093/rheumatology/ker269.
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66:940–4.
Wooten M. Systemic sclerosis and malignancy: a review of the literature. South Med J. 2008;101:59–62.
Tufanelli DL, Winkelmann R. Systemic scleroderma: a clinical study of 727 cases. Arch Dermatol. 1961;84:359–71.
Schady W, Sheard A, Hassell A, Holt L, Jayson MIV, Klimiuk P. Peripheral nerve dysfunction in scleroderma. Q J Med. 1991;80:661–75.
Averbuch-Heller L, Steiner I, Abramsky O. Neurologic manifestations of progressive systemic sclerosis. Arch Neurol. 1992;49:1292–5.
Hietaharju A, Jaaskelainen S, Kalimo H, Hietarinta M. Peripheral neuromuscular manifestations in systemic sclerosis (scleroderma). Muscle Nerve. 1993;16:1204–12.
Manneschi LI, Del Rosso A, Milia AF, Tani A, Nosi D, Pignone A, et al. Damage of cutaneous peripheral nervous system evolves differently according to the disease phase and subset of systemic sclerosis. Rheumatology. 2005;44:607–13.
Provitera V, Nolano M, Pappone N, di Girolamo C, Stancanelli A, Lullo F, et al. Distal degeneration of sensory and autonomic cutaneous nerve fibres in systemic sclerosis. Ann Rheum Dis. 2005;64:1524–6.
Leichenko T, Herrick AL, Alani SM, Hilton RC, Jayson MI. Mononeuritis in two patients with limited cutaneous systemic sclerosis. Br J Rheumatol. 1994;33:594–5.
Dyck PJ, Hunder GG, Dyck PJ. A case–control and nerve biopsy study of CREST multiple mononeuropathy. Neurology. 1997;49:1641–5.
Moore ME, Burke JM, Hartman JH, Korenzwitt E. Lumbosacral plexopathy in a woman with CREST syndrome and vasculitis. Arthritis Rheum. 1989;32:661–3.
Herrick AL, Oogarah P, Brammah TB, Freemont AJ, Jayson MI. Nervous system involvement in association with vasculitis and anticardiolipin antibodies in a patient with systemic sclerosis. Ann Rheum Dis. 1994;53:349–54.
Nitta Y, Sobue G. Progressive systemic sclerosis associated with multiple mononeuropathy. Dermatology. 1996;193:22–6.
Miller A, Ryan PF, Dowling JP. Vasculitis and thrombotic thrombocytopenic purpura in a patient with limited scleroderma. J Rheumatol. 1997;24:598–600.
Gordon RM, Silverstein A. Neurologic manifestations in progressive systemic sclerosis. Arch Neurol. 1970;22:126–34.
Lee P, Bruni J, Sukenik S. Neurological manifestations in systemic sclerosis (scleroderma). J Rheumatol. 1984;11:480–3.
Di Trapani G, Tulli A, LaCara A, Laurienzo P, Mazza S, David P. Peripheral neuropathy in course of progressive systemic sclerosis. Light and ultrastructural study. Acta Neuropathol. 1986;72:103–10.
Corbo M, Nemni R, Iannaccone S, Quattrini A, Lodi M, Praderio L, et al. Peripheral neuropathy in scleroderma. Clin Neuropathol. 1993;12:63–7.
Poncelet AN, Connolly MK. Peripheral neuropathy in scleroderma. Muscle Nerve. 2003;28:330–5.
Richter RB. Peripheral neuropathy and connective tissue disease. J Neuropathol Exp Neurol. 1954;13:168–80.
Nobuhara Y, Saito M, Goto R, Yoshidome Y, Kawamura M, Kasai T, et al. Chronic progressive sensory ataxic neuropathy associated with limited systemic sclerosis. J Neurol Sci. 2006;241:103–6.
Knupp-Oliveira S, Cerinic MM. Polyneuropathy as initial manifestation of systemic sclerosis (scleroderma). Scand J Rheumatol. 1999;28:260–1.
Mouthon L, Halimi C, Dussaule Md JC, Cayre-Castel Md M, Guillevin L. Brachial plexopathy associated with diffuse edematous scleroderma. Ann Med Interne (Paris). 2000;151:303–5.
Allanore Y, Zuber M, Kahan A. Brachial plexopathy associated with systemic sclerosis. Clin Rheumatol. 2002;21:401–2.
Andreadou E, Zouvelou V, Karandreas N, Kilidireas C. Anti-myelin-associated glycoprotein polyneuropathy coexistent with CREST syndrome. J Postgrad Med. 2012;58:57–9.
Machet L, Vaillant L, Machet MC, Esteve E, Muller C, Khallouf R, et al. Carpal tunnel syndrome and systemic sclerosis. Dermatology. 1992;185:101–3.
Al-Adhadh RN, Al-Sayed TA. Clinical features of systemic sclerosis. Saudi Med J. 2001;22:333–6.
Mondelli M, Romano C, Della Porta PD, Rossi A. Electrophysiological evidence of “nerve entrapment syndromes” and subclinical peripheral neuropathy in progressive systemic sclerosis (scleroderma). J Neurol. 1995;242:185–94.
Lori S, Matucci-Cerinic M, Casale R, et al. Peripheral nervous system involvement in systemic sclerosis: the median nerve as a target structure. Clin Exp Rheumatol. 1996;14:601–5.
Kaufman J, Canosos JJ. Progressive systemic sclerosis and meralgia paresthetica. Ann Intern Med. 1986;105:973.
Ko CY, Jones NF, Steen VD. Compression of the median nerve proximal to the carpal tunnel in scleroderma. J Hand Surg Am. 1996;21:363–5.
Tagliafico A, Panico N, Resmini E, Derchi LE, Ghio M, Martinoli C. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma). Eur J Radiol. 2011;77:377–82.
Farrell DA, Medsger Jr TA. Trigeminal neuropathy in progressive systemic sclerosis. Am J Med. 1982;73:57–62.
Teasdall RD, Frayha RA, Shulman LE. Cranial nerve involvement in systemic sclerosis (scleroderma): a report of 10 cases. Medicine. 1980;59:149–59.
Ribeiro RT, Fialho LM, de Souza LT, Barsottini OG. Trigeminal sensory neuropathy associated with systemic sclerosis: report of three Brazilian cases. Arq Neuropsiquiatr. 2009;67:494–5.
Lally EV, Jimenez SA. Vocal cord palsy in systemic sclerosis. J Rheumatol. 1988;15:1876–7.
Boschi A, Snyders B, Lambert M. Bilateral optic neuropathy associated with the CREST variant of scleroderma. Eur J Ophthalmol. 1993;3:219–22.
Shimohata T, Sato T, Akaiwa Y, Yamamoto Y, Yamamoto S, Suzuki M. Isolated trochlear nerve palsy in CREST syndrome. Eur Neurol. 2003;50:181–2.
Amor-Dorado JC, Arias-Nuñez MC, Miranda-Filloy JA, Gonzalez-Juanatey C, Llorca J, Gonzalez-Gay MA. Audiovestibular manifestations in patients with limited systemic sclerosis and centromere protein-B (CENP-B) antibodies. Medicine. 2008;87:131–41.
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40:1725.
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Collins, M.P., Kissel, J.T. (2014). Vasculitic Neuropathies and Neuropathies of Connective Tissue Diseases. In: Katirji, B., Kaminski, H., Ruff, R. (eds) Neuromuscular Disorders in Clinical Practice. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6567-6_36
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