Laboratory Abnormalities in Primary Sjögren’s Syndrome

  • Pilar Brito-Zerón
  • Roberto Pérez-Alvarez
  • Marta Pérez-de-Lis
  • Carmen Hidalgo-Tenorio
  • Manuel Ramos-Casals


Sjögren’s syndrome (SS) is a systemic autoimmune disease that presents with sicca symptomatology of the main mucosa surfaces. The spectrum of the disease extends from sicca syndrome to systemic involvement (extraglandular manifestations) and may be complicated by the development of lymphoma. Patients with SS may present with a broad spectrum of autoantibodies and laboratory abnormalities. Most of the laboratory abnormalities have been described more frequently in patients with positive immunological markers. Some may be the first manifestation of primary SS, while others have no clinical significance.


Aplastic Anemia Pernicious Anemia Chronic Atrophic Gastritis Monoclonal Immunoglobulin Sicca Syndrome 
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  1. 1.
    Ramos-Casals M, Font J, Garcia-Carrasco M, Brito MP, Rosas J, Calvo-Alen J, et al. Primary Sjogren syndrome: hematologic patterns of disease expression. Medicine (Baltimore). 2002;81:281–92.Google Scholar
  2. 2.
    Davidson BKS, Kelly CA, Griffiths ID. Primary Sjogren’s syndrome in the North East of England: a long-term follow-up study. Rheumatology. 1999;38:245–53.PubMedGoogle Scholar
  3. 3.
    Moutsopoulos HM, Steinberg AD, Fauci AS, Lane HC, Papadopoulos NM. High incidence of free monoclonal light chains in the sera of patients with Sjögren’s syndrome. J Immunol. 1983;130:2263–5.Google Scholar
  4. 4.
    Youinou P, Fauquert P, Pennec YL, Bendaoud B, Katsikis P, Le Goff P. Raised C-reactive protein response in rheumatoid arthritis patients with secondary Sjogren’s syndrome. Rheumatol Int. 1990;10:39–41.PubMedGoogle Scholar
  5. 5.
    Yazisiz V, Avci AB, Erbasan F, Kiris E, Terzioglu E. Diagnostic performance of minor salivary gland biopsy, serological and clinical data in Sjögren’s syndrome: a retrospective analysis. Rheumatol Int. 2009;29:403–9.PubMedGoogle Scholar
  6. 6.
    Kahn MF, Peltier AP, Meyer O, Piette JC Les Maladies systemiques. Gougerot Sjogren’s syndrome. Ed. Flammarion, Paris; 1991. pp. 517–8.Google Scholar
  7. 7.
    Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ. Sjogren’s syndrome a clinical, pathological and serological study of 62 cases. Medicine (Baltimore). 1965;44:187–231.Google Scholar
  8. 8.
    Martínez-Lavin M, Vaughan J, Tan E. Autoantibodies and the spectrum of Sjogren’s syndrome. Ann Intern Med. 1979;91:185–90.PubMedGoogle Scholar
  9. 9.
    Alexander EL, Arnett FC, Provost TT, Stevens MB. Sjogren’s syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperactivity. Ann Intern Med. 1983;98:155–9.PubMedGoogle Scholar
  10. 10.
    Sutcliffe N, Inanc M, Speight P, Isenberg D. Predictors of lymphoma development in primary Sjogren’s syndrome. Semin Arthritis Rheum. 1998;28:80–7.PubMedGoogle Scholar
  11. 11.
    Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum. 2000;29:296–304.PubMedGoogle Scholar
  12. 12.
    Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM. Hematological manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspects. Medicine (Baltimore). 2009;88:284–93.Google Scholar
  13. 13.
    Markusse HM, Veldhoven CH, Swaak AJ, Smeenk RT. The clinical significance of the detection of anti-Ro/SS-A and anti-La/SS-B autoantibodies using purified recombinant proteins in primary Sjögren’s syndrome. Rheumatol Int. 1993;13:147–50.PubMedGoogle Scholar
  14. 14.
    Amman AJ, Hong R. Selective immunoglobulinA deficiency: presentation of 30 cases and a review of the literature. Medicine (Baltimore). 1971;50:226–36.Google Scholar
  15. 15.
    Rodriguez-Cuartero A, Ceballos A, Gómez del Cerro A. Síndrome de Sjogren primario y deficiencia de immunoglobulinA. Rev Clin Esp. 1991;198:299–300.Google Scholar
  16. 16.
    Perez Pena F, Martinez Santos P, Sanchez Ramos A, Mateos Sanchez A, Lopez Alonso G. Deficiencia selectiva de immunoglobulinA. Rev Clin Esp. 1978;148:521–3.PubMedGoogle Scholar
  17. 17.
    Matter L, Wilhelm J, Angehrn W. Selective antibody deficiency and recurrent pneumococcal bacteremia in a patient with Sjogren’s syndrome: hyperimmunoglobulinemia G, and deficiencies of immunoglobulinG2 and immunoglobulinG4. N Engl J Med. 1985;312:1039–42.PubMedGoogle Scholar
  18. 18.
    Montecucco C, Cherie-Ligniere EL, Rosso R, Longhi M, Riccardi A. Sjogren-like syndrome in kappa chain deficiency. Arthritis Rheum. 1986;29:1532–3.PubMedGoogle Scholar
  19. 19.
    Eriksson P, Almroth G, Denneberg T, Lindstrom FD. ImmunoglobulinG2 deficiency in primary Sjogren’s syndrome and hypergammaglobulinemic purpura. Clin Immunol Immunopathol. 1994;70:60–5.PubMedGoogle Scholar
  20. 20.
    Steuer A, McCrea DJ, Colaco CB. Primary Sjogren’s syndrome, ulcerative colitis and selective immunoglobulinA deficiency. Postgrad Med J. 1996;72:499–500.PubMedGoogle Scholar
  21. 21.
    Wanchu A, Bambery P, Sud A, Chawla Y, Vaiphei K, Deodhar SD. Autoimmune hepatitis in a patient with primary Sjogren’s syndrome and selective immunoglobulinA deficiency. Trop Gastroenterol. 1998;19:62–3.PubMedGoogle Scholar
  22. 22.
    Sugai S, Shimizu S, Konda S. Lymphoproliferative disorders in Japanese patients with Sjogren’s syndrome. Scand J Rheumatol Suppl. 1986;61:118–22.PubMedGoogle Scholar
  23. 23.
    Walters MT, Stevenson FK, Herbert A, Cawley MI, Smith JL. Lymphoma in Sjogren’s syndrome: urinary monoclonal free light chains as a diagnostic aid and a means of tumour monitoring. Scand J Rheumatol Suppl. 1986;61:114–7.PubMedGoogle Scholar
  24. 24.
    Pariente D, Anaya JM, Combe B, Jorgensen C, Emberger JM, Rossi JF, et al. Non-Hodgkin lymphoma associated with primary Sjogren’s syndrome. Eur J Med. 1992;1:337–42.PubMedGoogle Scholar
  25. 25.
    Sibilia J, Cohen-Solal J. Prevalence of monoclonal gammopathy and myeloma in a cohort of primary Sjögren’ syndrome. Arthritis Rheum. 1999;42 Suppl 9:S140. abstract.Google Scholar
  26. 26.
    Brito-Zerón P, Ramos-Casals M, Nardi N, Cervera R, Yagüe J, Ingelmo M, et al. Circulating monoclonal immunoglobulins in Sjögren syndrome. Medicine (Baltimore). 2005;84:90–7.Google Scholar
  27. 27.
    Ramos-Casals M, Cervera R, Yagüe J, García-Carrasco M, Trejo O, Jiménez S, et al. Cryoglobulinemia in primary SS: prevalence and clinical characteristics in a series of 115 patients. Semin Arthritis Rheum. 1998;28:200–5.PubMedGoogle Scholar
  28. 28.
    De Re V, De Vita S, Gasparotto D, Marzotto A, Carbone A, Ferraccioli G, et al. Salivary gland B cell lymphoproliferative disorders in Sjogren’s syndrome present a restricted use of antigen receptor gene segments similar to those used by hepatitis C virus-associated non Hodgkins’s lymphomas. Eur J Immunol. 2002;32:903–10.PubMedGoogle Scholar
  29. 29.
    Michalski JP, Daniels TE, Talal N, Grey HM. β2 microglobulin and lymphocytic infiltration in Sjogren’s syndrome. N Engl J Med. 1975;293:1228–31.PubMedGoogle Scholar
  30. 30.
    Bethea M, Forman DT. Beta 2-microglobulin: its significance and clinical usefulness. Ann Clin Lab Sci. 1990;20:163–8.PubMedGoogle Scholar
  31. 31.
    Bianucci G, Campana G, Bongi SM, Palermo C, D’Agata A. Salivary and serum beta 2-microglobulin in the diagnosis of primary Sjögren syndrome. Minerva Med. 1992;83:705–13.PubMedGoogle Scholar
  32. 32.
    Maddali Bongi S, Campana G, D’Agata A, Palermo C, Bianucci G. The diagnosis value of beta 2-microglobulin and immunoglobulins in primary Sjögren’s syndrome. Clin Rheumatol. 1995;14:151–6.PubMedGoogle Scholar
  33. 33.
    Mogi M, Kage T, Chino T, Yoshitake K, Harada M. Increased beta 2-microglobulin in both parotid and submandibular/sublingual saliva from patients with Sjögren’s syndrome. Arch Oral Biol. 1994;39:913–5.PubMedGoogle Scholar
  34. 34.
    Van der Geest SA, Markusse HM, Swaak AJ. Beta 2 microglobulin measurements in saliva of patients with primary Sjögren’s syndrome: influence of flow. Ann Rheum Dis. 1993;52:461–3.PubMedGoogle Scholar
  35. 35.
    Markusse HM, Huysen JC, Nieuwenhuys EJ, Swaak AJ. Beta 2 microglobulin in tear fluid from patients with primary Sjögren’s syndrome. Ann Rheum Dis. 1992;51:503–5.PubMedGoogle Scholar
  36. 36.
    Ström T, Evrin PE, Karlsson A. Serum beta 2-microglobulin in Sjögren’s syndrome. Scand J Rheumatol. 1978;7:97–100.PubMedGoogle Scholar
  37. 37.
    Du D, Pu Y, Zhao Y. The value of salivary beta 2 microglobulin concentration for diagnosis of Sjögren’s syndrome. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 1997;19:72–4.PubMedGoogle Scholar
  38. 38.
    Krejsek J, Slezák R, Kopecky O, Derner V, Andrys C. Elevation of serum soluble intercellular adhesion molecule-1 (sICAM-1) and beta 2-microglobulin in Sjögren’s syndrome. Clin Rheumatol. 1997;16:149–53.PubMedGoogle Scholar
  39. 39.
    Pertovaara M, Korpela M, Uusitalo H, Pukander J, Miettinen A, Helin H, et al. Clinical follow up study of 87 patients with sicca symptoms (dryness of eyes or mouth, or both). Ann Rheum Dis. 1999;58:423–7.PubMedGoogle Scholar
  40. 40.
    Bianucci G, Campana G, Maddali-Bongi S, D’Agata A, Pradella F, Colafranceschi M, et al. Serum beta 2-microglobulin and HLA alloantigens in primary Gougerot-Sjogren syndrome: a possible relation with HLA-DR3 specificity. Rev Rhum Mal Osteoartic. 1991;58:339–42.PubMedGoogle Scholar
  41. 41.
    Gottenberg JE, Busson M, Cohen-Solal J, Lavie F, Abbed K, Kimberly RP, et al. Correlation of serum B lymphocyte stimulator and beta 2 microglobulin with autoantibody secretion and systemic involvement in primary Sjögren’s syndrome. Ann Rheum Dis. 2005;64:1050–5.PubMedGoogle Scholar
  42. 42.
    Lahdensuo A, Korpela M. Pulmonary findings in patients with primary Sjogren’s syndrome. Chest. 1995;102:316–9.Google Scholar
  43. 43.
    Hatron PY, Wallaert B, Gosset D, Tonnel AB, Gosselin B, Voisin C, et al. Subclinical lung inflammation in primary Sjögren’s syndrome: relationship between bronchoalveolar lavage cellular analysis finding and characteristics of the disease. Arthritis Rheum. 1987;30:1226–31.PubMedGoogle Scholar
  44. 44.
    Pertovaara M, Korpela M, Kouri T, Pasternack A. The occurrence of renal involvement in primary Sjogren’s syndrome: a study of 78 patients. Rheumatology. 1999;38:1113–20.PubMedGoogle Scholar
  45. 45.
    Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A. A longitudinal cohort study of Finnish patients with primary Sjögren’s syndrome: clinical, immunological and epidemiological aspects. Ann Rheum Dis. 2001;60:467–72.PubMedGoogle Scholar
  46. 46.
    Benucci M, Li Gobbi F, Del Gobbo A, Gambacorta G, Mannoni A. [Association between serum amyloid A (SAA) in salivary glands and high levels of circulating beta 2-microglobulin in patients with Sjögren syndrome]. Reumatismo. 2003;55:98–101.PubMedGoogle Scholar
  47. 47.
    Ramakrishna R, Chaudhuri K, Sturgess A, Manoharan A. Haematological manifestations of primary Sjogren’s syndrome: a clinicopathological study. Q J Med. 1992;84:547–54.Google Scholar
  48. 48.
    Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, del Pino-Montes J, et al. Primary Sjögren syndrome in Spain: clinical and immunological expression in 1010 patients. Medicine (Baltimore). 2008;87:210–9.Google Scholar
  49. 49.
    Baboonian C, Venables PJW, Booth J, Williams DG, Roffe LM, Maini RN. Virus infection induces redistribution and membrane localisation of the nuclear antigen La (SSB): a possible mechanism for autoimmunity. Clin Exp Immunol. 1989;78:454–9.PubMedGoogle Scholar
  50. 50.
    Le Feber WP, Norris DA, Ryan S, Huff DC, Lee LA, Kubo M, et al. Ultraviolet light induced expression of selected nuclear antigens on cultured human keratinocytes. J Clin Invest. 1984;74:1545–51.Google Scholar
  51. 51.
    Ramos-Casals M, Cervera R, García-Carrasco M, Vidal J, Trejo O, Jimenez S, et al. Cytopenia and past human parvovirus B19 infection in patients with primary Sjögren’s syndrome. Semin Arthritis Rheum. 2000;29:373–8.PubMedGoogle Scholar
  52. 52.
    Schattner A, Friedman J, Klepfish A, Berrebi A. Immune cytopenias as the presenting finding in primary Sjogren’s syndrome. Q J Med. 2000;93:825–9.Google Scholar
  53. 53.
    Chudwin DS, Daniels TE, Wara DW, Ammann AJ, Barrett DJ, Whitcher JP, et al. Spectrum of Sjogren’s syndrome in children. J Pediatr. 1981;98:213–7.PubMedGoogle Scholar
  54. 54.
    Boling EP, Wen J, Reveille JD, Bias WB, Chused TM, Arnett FC. Primary Sjogren’s syndrome and autoimmune hemolytic anemia in sisters. Am J Med. 1983;74:1066–71.PubMedGoogle Scholar
  55. 55.
    Schattner A, Shtalrid M, Berrebi A. Autoimmune hemolytic anemia preceding Sjogren’s syndrome. J Rheumatol. 1983;10:482–4.PubMedGoogle Scholar
  56. 56.
    de la Montane Roque P, Arlet P, Chartier JP, Cornu JJ, Juchet H, Ollier S, et al. Autoimmune hemolytic anemia disclosing primary Gougerot-Sjogren syndrome. Rev Med Interne. 1993;14:133–4.Google Scholar
  57. 57.
    Usui K, Anzai C, Sano K. Primary Sjogren’s syndrome with pulmonary hypertension. Nihon Koyuki Gakkai Zasshi. 1998;36:478–81.Google Scholar
  58. 58.
    Fye KH, Daniels TE, Zulman J, Michalski JP, Jaffe R, Talal N. Aplastic anemia and lymphoma in Sjogren’s syndrome. Arthritis Rheum. 1980;23:1321–5.PubMedGoogle Scholar
  59. 59.
    Yoshida H, Wakashin M, Okuda K. Successful treatment of aplastic anemia associated with chronic thyroiditis and Sjogren’s syndrome. J Rheumatol. 1986;13:1189–90.PubMedGoogle Scholar
  60. 60.
    Quiquandon I, Morel P, Lai JL, Bauters F, Dresch C, Gluckman E, et al. Primary Sjogren’s syndrome and aplastic anaemia. Ann Rheum Dis. 1997;56:438–41.PubMedGoogle Scholar
  61. 61.
    Williamson J, Paterson RW, McGavin DD, Greig WR, Whaley K. Sjogren’s syndrome in relation to pernicious anaemia and idiopathic Addison’s disease. Br J Ophthalmol. 1970;54:31–6.PubMedGoogle Scholar
  62. 62.
    Wegelious O, Fyhrquist F, Adner PL. Sjogren’s syndrome associated with vitamin B12 deficiency. Acta Rheumatol Scand. 1970;16:184–90.Google Scholar
  63. 63.
    Pedro-Botet J, Coll J, Tomas S, Soriano JC, Gutierrez-Cebollada J. Primary Sjogren’s syndrome associated with chronic atrophic gastritis and pernicious anemia. J Clin Gastroenterol. 1993;16:146–8.PubMedGoogle Scholar
  64. 64.
    Koramaz I, Sonmez M, Pulathan Z, Cobanoglu U, Karti SS. Successful coronary artery bypass grafting in a patient with aplastic anemia and Sjögren syndrome. Saudi Med J. 2006;27:1251–2.PubMedGoogle Scholar
  65. 65.
    Matsumoto N, Kagawa H, Ichiyoshi H, Iguchi T, Yamanaka Y, Kishimoto Y, et al. Aplastic anemia complicating Sjögren’s syndrome. Intern Med. 1997;36:371–4.PubMedGoogle Scholar
  66. 66.
    Satoh M, Yamagata H, Watanabe F, Matsushita Y, Nakayama S, Murakami M, et al. A case of Sjögren’s syndrome complicating immune-mediated aplastic anemia. Clin Rheumatol. 1993;12:257–60.PubMedGoogle Scholar
  67. 67.
    Giordano N, Senesi M, Battisti E, DeRegis FM, Gennari C. Sjogren’s syndrome and pure red cell aplasia. Clin Exp Rheumatol. 1996;14:344–5.PubMedGoogle Scholar
  68. 68.
    García-García C, Jaén-Aguila F, Hidalgo-Tenorio C. Jiménez-Alonso JF [Pure red cell aplasia as first manifestation of primary Sjögren syndrome]. Rev Clin Esp. 2009;209:203–4.PubMedGoogle Scholar
  69. 69.
    Cavazzana I, Ceribelli A, Franceschini F, Cattaneo R. Unusual association between pure red cell aplasia and primary Sjögren’s syndrome: a case report. Clin Exp Rheumatol. 2007;25: 309–11.PubMedGoogle Scholar
  70. 70.
    Assimakopoulos SF, Michalopoulou S, Melachrinou M, Giannakoulas N, Papakonstantinou C, Lekkou A, et al. Primary Sjögren syndrome complicated by autoimmune haemolytic anemia and pure red cell aplasia. Am J Med Sci. 2007;334:493–6.PubMedGoogle Scholar
  71. 71.
    Fujiu K, Kanno R, Shio Y, Ohsugi J, Nozawa Y, Gotoh M. Triad of thymoma, myasthenia gravis and pure red cell aplasia combined with Sjögren’s syndrome. Jpn J Thorac Cardiovasc Surg. 2004;52:345–8.PubMedGoogle Scholar
  72. 72.
    Ergas D, Tsimanis A, Shtalrid M, Duskin C, Berrebi A. T-gamma large granular lymphocyte leukaemia associated with a megakaryocytic thrombocytopenic purpura, Sjögren’s syndrome and polyglandular autoimmune syndrome type II, with subsequent development of pure red cell aplasia. Am J Hematol. 2002;69:132–4.PubMedGoogle Scholar
  73. 73.
    Ibkhatra S, Jacobson L, Manthorpe R. The association of pure red cell aplasia and primary Sjogren’s syndrome. Clin Exp Rheumatol. 1997;15:119–20.PubMedGoogle Scholar
  74. 74.
    Bouali F, Berrah A, Si Ahmed-Bouali D, Harrieche F, Benhalima M, Hamladji RM, et al. [Immunological abnormalities in myelodysplastic syndromes. Prospective study (series of 40 patients)]. Rev Med Interne. 2005;26:777–83.PubMedGoogle Scholar
  75. 75.
    Roy-Peaud F, Paccalin M, Le Moal G, Landron C, Juhel L, Roblot P, et al. [Association of systemic disease and myelodysplastic syndromes: a retrospective study of 14 cases]. Presse Med. 2003;32:538–43.PubMedGoogle Scholar
  76. 76.
    DeCoteau WE, Katakkar SB, Skinnider L, Hayton RC, Somerville EA. Sjögren’s syndrome terminating as a myeloproliferative disorder. J Rheumatol. 1975;2:331–5.PubMedGoogle Scholar
  77. 77.
    Castro M, Conn DL, Su WP, Garton JP. Rheumatic manifestations in myelodysplastic syndromes. J Rheumatol. 1991;18:721–7.PubMedGoogle Scholar
  78. 78.
    Rodriguez-Cuartero A, Pérez-Blanco FJ, Urbano-Jiménez F. Sjögren’s syndrome and pernicious anaemia. Scand J Rheumatol. 1998;27:83–5.PubMedGoogle Scholar
  79. 79.
    James JM, Zittoun R, Simon F, Slama G, Facquet-Danis J, Bilski-Pasquier G. Pernicious anaemia associated with Hashimoto’s thyroiditis, Sjögren’s syndrome and chondrocalcinosis. Sem Hop. 1978;54:1041–4.PubMedGoogle Scholar
  80. 80.
    Zittoun R, Debain P, James JM, Bilski-Pasquier G. Hematological manifestations and complications in Sjögren’s syndrome. Sem Hop. 1978;54:1011–20.PubMedGoogle Scholar
  81. 81.
    Harley JB, Alexander EL, Bias WB, Fox OF, Provost TT, Reichlin M, et al. Anti-Ro(SS-A) and anti-La(SS-B) in patients with Sjogren’s syndrome. Arthritis Rheum. 1986;29:196–206.PubMedGoogle Scholar
  82. 82.
    Brito-Zerón P, Soria N, Muñoz S, Bové A, Akasbi M, Berenguer R, et al. Prevalence and clinical relevance of autoimmune neutropenia in patients with primary Sjögren’s syndrome. Semin Arthritis Rheum. 2009;38:389–95.PubMedGoogle Scholar
  83. 83.
    Aoki A, Ohno S, Ueda A. Hematological abnormalities of primary Sjogren’s syndrome. Nihon Rinsho Meneki Gakkai Kaishi. 2000;23:124–8.PubMedGoogle Scholar
  84. 84.
    Kirtava Z, Blomberg J, Bredberg A, Henriksson G, Jacobsson L, Manthorpe R. CD4+ T-lymphocytopenia without HIV infection: increased prevalence among patients with primary Sjögren’s syndrome. Clin Exp Rheumatol. 1995;13:609–16.PubMedGoogle Scholar
  85. 85.
    Mandl T, Bredberg A, Jacobsson LT, Manthorpe R, Henriksson G. CD4+ T-lymphocytopenia – a frequent finding in anti-SSA antibody seropositive patients with primary Sjögren’s syndrome. J Rheumatol. 2004;31:726–8.PubMedGoogle Scholar
  86. 86.
    Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjogren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006;65:796–803.PubMedGoogle Scholar
  87. 87.
    Coppo P, Sibilia J, Maloisel F, et al. Primary Sjogren’s syndrome associated agranulocytosis: a benign disorder? Ann Rheum Dis. 2003;62:476–8.PubMedGoogle Scholar
  88. 88.
    Friedman J, Klepfish A, Miller EB, Ognenovski V, Ike RW, Schattner A. Agranulocytosis in Sjogren’s syndrome: two case reports and analysis of 11 additional reported cases. Semin Arthritis Rheum. 2002;31:338–45.PubMedGoogle Scholar
  89. 89.
    Gottenberg JE, Busson M, Loiseau P, et al. In primary Sjogren’s syndrome, HLA class II is associated exclusively with autoantibody production and spreading of the autoimmune response. Arthritis Rheum. 2003;48:2240–5.PubMedGoogle Scholar
  90. 90.
    Addas-Carvalho M, de Paula EV, Lima CS, Saad ST. Polymorphisms of interleukin-1 gene complex, IL6 and tumour necrosis factor genes in chronic idiopathic neutropenia of adults. Ann Hematol. 2005;84:709–14.PubMedGoogle Scholar
  91. 91.
    Gavrikova N, Zeidler C, Stanulla M, Germeshausen M, Schwinzer B, Welte K. TNF and lymphotoxin-alpha polymorphisms in patients with severe chronic neutropenia. Int J Hematol. 2001;74:477–8.PubMedGoogle Scholar
  92. 92.
    Neth OW, Bajaj-Elliott M, Turner MW, Klein NJ. Susceptibility to infection in patients with neutropenia: the role of the innate immune system. Br J Haematol. 2005;129:713–22.PubMedGoogle Scholar
  93. 93.
    Ramos-Casals M, Loustaud-Ratti V, De Vita S, SS-HCV Study Group, et al. Sjogren syndrome associated with hepatitis C virus: a multicenter analysis of 137 cases. Medicine (Baltimore). 2005;84:81–9.Google Scholar
  94. 94.
    Lamy T, Dauriac C, MoriceP LePrisePY. Agranulocytose et connectivites distincttes due syndrome de Felty: 4 observations, succes dans un cas de la ciclosporine. Rev Med Interne. 1990;11:325–8.PubMedGoogle Scholar
  95. 95.
    Shastri KA, Logue GL. Autoimmune neutropenia. Blood. 1993;81:1984–95.PubMedGoogle Scholar
  96. 96.
    Bux J, Behrens G, Jaeger G, Welte K. Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood. 1998;91:181–6.PubMedGoogle Scholar
  97. 97.
    Vivancos J, Vila M, Serra A, Loscos J, Anguita A. Failure of G-CSF therapy in neutropenia associated with Sjogren’s syndrome. Rheumatology (Oxford). 2002;41:471–3.Google Scholar
  98. 98.
    Friedman J, Schattner A, Shvidel L, Berrebi A. Characterization of T-cell large granular lymphocyte leukemia associated with Sjogren’s syndrome-an important but under-recognized association. Semin Arthritis Rheum. 2006;35:306–11.PubMedGoogle Scholar
  99. 99.
    Boros P, Odin JA, Chen J, Unkeless JC. Specificity and class distribution of FcγR-specific autoantibodies in patients with autoimmune disease. J Immunol. 1994;152:302.PubMedGoogle Scholar
  100. 100.
    Yamato E, Fujioka Y, Masugi F, Nakamaru M, Tahara Y, Kurata Y, et al. Autoimmune neutropenia with anti-neutrophil autoantibody associated with Sjogren’s syndrome. Am J Med Sci. 1990;300:102–3.PubMedGoogle Scholar
  101. 101.
    Lamour A, Le Corre R, Pennec YL, Cartron J, Youinou P. Heterogeneity of neutrophil antibodies in patients with primary Sjogren’s syndrome. Blood. 1995;86:3553–9.PubMedGoogle Scholar
  102. 102.
    Berrebi A, Shtalrid M, Talmor M, Vorst E. Thrombocytopenia in Sjogren’s syndrome. Arthritis Rheum. 1982;25:1510.PubMedGoogle Scholar
  103. 103.
    Sugai S, Shimizu S, Tachibana J, Sawada M, Hirose Y, Takiguchi T, et al. Monoclonal gammopathies in patients with Sjogren’s syndrome. Jpn J Med. 1988;27:2–9.PubMedGoogle Scholar
  104. 104.
    Choung BS, Yoo WH. Successful treatment with intravenous immunoglobulin of severe thrombocytopenia complicated in primary Sjögren’s syndrome. Rheumatol Int. 2010;(Epub ahead of print).Google Scholar

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© Springer-Verlag London Limited 2011

Authors and Affiliations

  • Pilar Brito-Zerón
    • 1
  • Roberto Pérez-Alvarez
    • 2
  • Marta Pérez-de-Lis
    • 2
  • Carmen Hidalgo-Tenorio
    • 3
  • Manuel Ramos-Casals
    • 1
  1. 1.Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune DiseasesHospital ClínicBarcelonaSpain
  2. 2.Department of Internal MedicineHospital MeixoeiroVigoSpain
  3. 3.Department of Internal MedicineHospital Virgen de las NievesGranadaSpain

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