Medium- and Small-Vessel Vasculitis

  • Rafael G. GrauEmail author


As a group, the systemic vasculitides are surprisingly common in the elderly population. Although large vessel vasculitis such as temporal arteritis or giant cell arteritis, is a more common form, medium- and small vessel vasculitis are more frequently being recognized. Morbidity and mortality is higher in this group as compared to large vessel vasculitis. The clinical features, diagnosis, and management of this group are discussed based on presently available information.


Vasculitis Aging Immunosuppression Giant cell arteritis Antineutrophil cytoplasmic antibodies Wegener’s granulomatosis Churg–Strauss syndrome Microscopic polyangiitis ANCA-associated renal ­vasculitis Leukocytoclastic vasculitis 


  1. 1.
    Hunder GG, Arend WP, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum. 1990;33(8):1065–7.PubMedCrossRefGoogle Scholar
  2. 2.
    Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–92.PubMedCrossRefGoogle Scholar
  3. 3.
    Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000;43(2):414–9.PubMedCrossRefGoogle Scholar
  4. 4.
    Gonzalez-Gay MA, Garcia-Porrua C. Systemic vasculitis in adults in northwestern Spain, 1988–1997. Clinical and epidemiologic aspects. Medicine. 1999;78(5):292–308.PubMedCrossRefGoogle Scholar
  5. 5.
    Panagiotakis SH, Perysinakis GS, Kritikos H, et al. The epidemiology of primary systemic vasculitides involving small vessels in Crete (southern Greece): a comparison of older versus younger adult patients. Clin Exp Rheumatol. 2009;27(3):409–15.PubMedGoogle Scholar
  6. 6.
    Takala JH, Kautiainen H, Leirisalo-Repo M. Survival of patients with Wegener’s granulomatosis diagnosed in Finland in 1981–2000. Scand J Rheumatol. 2010;39(1):71–6.PubMedCrossRefGoogle Scholar
  7. 7.
    Hoganson DD, From AM, Michet CJ. ANCA vasculitis in the elderly. J Clin Rheumatol. 2008;14(2):78–81.PubMedCrossRefGoogle Scholar
  8. 8.
    Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117(1):39–50.PubMedCrossRefGoogle Scholar
  9. 9.
    van der Woude FJ, Rasmussen N, Lobatto S, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet. 1985;1(8426):425–9.PubMedCrossRefGoogle Scholar
  10. 10.
    Falk RJ, Jennette JC. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med. 1988;318(25):1651–7.PubMedCrossRefGoogle Scholar
  11. 11.
    Kerr GS, Fleisher TA, Hallahan CW, Leavitt RY, Fauci AS, Hoffman GS. Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegener’s granulomatosis. Arthritis Rheum. 1993;36(3):365–71.PubMedCrossRefGoogle Scholar
  12. 12.
    Juby AG, Davis P, McElhaney JE, Gravenstein S. Prevalence of selected autoantibodies in different elderly subpopulations. Br J Rheumatol. 1994;33(12):1121–4.PubMedCrossRefGoogle Scholar
  13. 13.
    Maillefert JF, Pfitzenmeyer P, Thenet M, et al. Prevalence of ANCA in a hospitalized elderly French population. Clin Exp Rheumatol. 1997;15(6):603–7.PubMedGoogle Scholar
  14. 14.
    Moscardi F, Ianiro JL, Maxit MJ. Antineurotrophil cytoplasmic antibodies (ANCA) in the elderly. Medicina. 1997;57(1):36–40.PubMedGoogle Scholar
  15. 15.
    Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488–98.PubMedGoogle Scholar
  16. 16.
    Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 1021;43(5):1021–32.CrossRefGoogle Scholar
  17. 17.
    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(1):76–85.PubMedGoogle Scholar
  18. 18.
    Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349(1):36–44.PubMedCrossRefGoogle Scholar
  19. 19.
    Langford CA, Talar-Williams C, Barron KS, Sneller MC. Use of a cyclophosphamide-induction methotrexate-maintenance regimen for the treatment of Wegener’s granulomatosis: extended follow-up and rate of relapse. Am J Med. 2003;114(6):463–9.PubMedCrossRefGoogle Scholar
  20. 20.
    Langford CA, Talar-Williams C, Sneller MC. Mycophenolate mofetil for remission maintenance in the treatment of Wegener’s granulomatosis. Arthritis Rheum. 2004;51(2):278–83.PubMedCrossRefGoogle Scholar
  21. 21.
    Keogh KA, Wylam ME, Stone JH, Specks U. Induction of remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(1):262–8.PubMedCrossRefGoogle Scholar
  22. 22.
    Weiner SR, Paulus HE, Weisbart RH. Wegener’s granulomatosis in the elderly. Arthritis Rheum. 1986;29(9):1157–9.PubMedCrossRefGoogle Scholar
  23. 23.
    Krafcik SS, Covin RB, Lynch 3rd JP, Sitrin RG. Wegener’s granulomatosis in the elderly. Chest. 1996;109(2):430–7.PubMedCrossRefGoogle Scholar
  24. 24.
    Huong Du LT, Wechsler B, Piette JC, et al. [Wegener’s granulomatosis in elderly subjects. 37 cases]. Presse Med. 1988;17(45):2379–82.Google Scholar
  25. 25.
    Turcu A, Bielefeld P, Besancenot J-F, Lorcerie B, Pfitzenmeyer P. Vasculitis in the very elderly. Gerontology. 2002;48(3):174–8.PubMedCrossRefGoogle Scholar
  26. 26.
    Parry R, Sherwin S, Fletcher V, Medcalf P. ANCA-associated vasculitis: diagnosis and treatment in the elderly. Postgrad Med J. 1996;72(849):423–6.PubMedCrossRefGoogle Scholar
  27. 27.
    Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999;78(1):26–37.CrossRefGoogle Scholar
  28. 28.
    Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984;63(2):65–81.Google Scholar
  29. 29.
    Guillevin L, Visser H, Noel LH, et al. Antineutrophil cytoplasm antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome – 62 patients. J Rheumatol. 1993;20(8):1345–9.PubMedGoogle Scholar
  30. 30.
    Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med. 1985;56(220):467–83.PubMedGoogle Scholar
  31. 31.
    Pagnoux C. Clinical features and outcomes in 345 patients with polyarteritis nodosa. Arthritis Rheum. 2010;62(2):616–26.PubMedCrossRefGoogle Scholar
  32. 32.
    Gayraud M, Guillevin L, le Toumelin P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001;44(3):666–75.PubMedCrossRefGoogle Scholar
  33. 33.
    Puisieux F, Woesteland H, Hachulla E, Hatron PY, Dewailly P, Devulder B. Clinical symptomatology and prognosis of periarteritis nodosa in the elderly. Retrospective study of 25 periarteritis nodosa cases in young adults and 22 cases in aged patients. Rev Med Interne. 1997;18(3):195–200.PubMedCrossRefGoogle Scholar
  34. 34.
    Mouthon L, Le Toumelin P, Andre MH, Gayraud M, Casassus P, Guillevin L. Polyarteritis nodosa and Churg-Strauss angiitis: characteristics and outcome in 38 patients over 65 years. Medicine (Baltimore). 2002;81(1):27–40.CrossRefGoogle Scholar
  35. 35.
    Bindi P, Mougenot B, Mentre F, et al. Necrotizing crescentic glomerulonephritis without significant immune deposits: a clinical and serological study. Q J Med. 1993;86(1):55–68.PubMedGoogle Scholar
  36. 36.
    Garrett PJ, Dewhurst AG, Morgan LS, Mason JC, Dathan JR. Renal disease associated with circulating antineutrophil cytoplasm activity. Q J Med. 1992;85(306):731–49.PubMedGoogle Scholar
  37. 37.
    Satchell SC, Nicholls AJ, D’Souza RJ, Beaman M. Renal vasculitis: increasingly a disease of the elderly? Nephron. 2004;97(4):c142–6.PubMedGoogle Scholar
  38. 38.
    Haas M, Spargo BH, Wit EJ, Meehan SM. Etiologies and outcome of acute renal insufficiency in older adults: a renal biopsy study of 259 cases. Am J Kidney Dis. 2000;35(3):433–47.PubMedCrossRefGoogle Scholar
  39. 39.
    Harper L, Savage CO. ANCA-associated renal vasculitis at the end of the twentieth century – a disease of older patients. Rheumatology (Oxford). 2005;44(4):495–501.CrossRefGoogle Scholar
  40. 40.
    Higgins RM, Goldsmith DJ, Connolly J, et al. Vasculitis and rapidly progressive glomerulonephritis in the elderly. Postgrad Med J. 1996;72(843):41–4.PubMedCrossRefGoogle Scholar
  41. 41.
    Hauer HA, Bajema IM, Van Houwelingen HC, et al. Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int. 2002;62(5):1732–42.PubMedCrossRefGoogle Scholar
  42. 42.
    Chung JB, Armstrong K, Schwartz JS, Albert D. Cost-effectiveness of prophylaxis against Pneumocystis carinii pneumonia in patients with Wegner’s granulomatosis undergoing immunosuppressive therapy. Arthritis Rheum. 2000;43(8):1841–8.PubMedCrossRefGoogle Scholar
  43. 43.
    Haubitz M, Bohnenstengel F, Brunkhorst R, Schwab M, Hofmann U, Busse D. Cyclophosphamide pharmacokinetics and dose requirements in patients with renal insufficiency. Kidney Int. 2002;61(4):1495–501.PubMedCrossRefGoogle Scholar
  44. 44.
    Iglesias-Gamarra A, Restrepo JF, Matteson EL. Small-vessel vasculitis. Curr Rheumatol Rep. 2007;9(4):304–11.PubMedCrossRefGoogle Scholar
  45. 45.
    Garcia-Porrua C, Gonzalez-Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schonlein purpura in adults. Semin Arthritis Rheum. 1999;28(6):404–12.PubMedCrossRefGoogle Scholar
  46. 46.
    Diehl MP, Harrington T, Olenginski T. Elderly-onset Henoch-Schonlein purpura: a case series and review of the literature. J Am Geriatr Soc. 2008;56(11):2157–9.PubMedCrossRefGoogle Scholar
  47. 47.
    Ferri C, Sebastiani M, Giuggioli D, et al. Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients. Semin Arthritis Rheum. 2004;33(6):355–74.PubMedCrossRefGoogle Scholar
  48. 48.
    Trejo O, Ramos-Casals M, Garcia-Carrasco M, et al. Cryoglobulinemia: study of etiologic factors and clinical and immunologic features in 443 patients from a single center. Medicine (Baltimore). 2001;80(4):252–62.CrossRefGoogle Scholar
  49. 49.
    Langford CA. Small-vessel vasculitis: therapeutic management. Curr Rheumatol Rep. 2007;9(4):328–35.PubMedCrossRefGoogle Scholar
  50. 50.
    Langford CA. Vasculitis in the geriatric population. [Review] [110 refs]. Rheum Dis Clin North Am. 2007;33(1):177–95.PubMedCrossRefGoogle Scholar
  51. 51.
    Fehrman-Ekholm I, Skeppholm L. Renal function in the elderly (>70 years old) measured by means of iohexol clearance, serum creatinine, serum urea and estimated clearance. Scand J Urol Nephrol. 2004;38(1):73–7.PubMedCrossRefGoogle Scholar
  52. 52.
    Kohli HS, Jairam A, Bhat A, et al. Safety of kidney biopsy in elderly: a prospective study. Int Urol Nephrol. 2006;38(3–4):815–20.PubMedGoogle Scholar
  53. 53.
    de Lind van Wijngaarden RAF, Hauer HA, Wolterbeek R, et al. Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: a prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol. 2006;17(8):2264–74.PubMedCrossRefGoogle Scholar
  54. 54.
    Janssens JP. Aging of the respiratory system: impact on pulmonary function tests and adaptation to exertion. [Review] [128 refs]. Clin Chest Med. 2005;26(3):469–84.PubMedCrossRefGoogle Scholar
  55. 55.
    Nasr SH, D’Agati VD, Said SM, et al. Pauci-immune crescentic glomerulonephritis superimposed on diabetic glomerulosclerosis. Clin J Am Soc Nephrol. 2008;3(5):1282–8 [Erratum appears in Clin J Am Soc Nephrol. 2009 Feb;4(2):516].PubMedCrossRefGoogle Scholar
  56. 56.
    Weidanz F, Day CJ, Hewins P, Savage CO, Harper L. Recurrences and infections during continuous immunosuppressive therapy after beginning dialysis in ANCA-associated vasculitis. Am J Kidney Dis. 2007;50(1):36–46.PubMedCrossRefGoogle Scholar
  57. 57.
    Charlier C, Henegar C, Launay O, et al. Risk factors for major infections in Wegener granulomatosis: analysis of 113 patients. Ann Rheum Dis. 2009;68(5):658–63.PubMedCrossRefGoogle Scholar
  58. 58.
    Matteson EL, Gold KN, Bloch DA, Hunder GG. Long-term survival of patients with Wegener’s granulomatosis from the American College of Rheumatology Wegener’s Granulomatosis Classification Criteria Cohort. Am J Med. 1996;101(2):129–34.PubMedCrossRefGoogle Scholar
  59. 59.
    Bijlsma JWJ, Boers M, Saag KG, Furst DE. Glucocorticoids in the treatment of early and late RA. Ann Rheum Dis. 2003;62(11):1033–7.PubMedCrossRefGoogle Scholar
  60. 60.
    Nesher G, Sonnenblick M, Friedlander Y. Analysis of steroid related complications and mortality in temporal arteritis: a 15-year survey of 43 patients. J Rheumatol. 1994;21(7):1283–6.PubMedGoogle Scholar
  61. 61.
    Gabriel SE, Sunku J, Salvarani C, O’Fallon WM, Hunder GG. Adverse outcomes of antiinflammatory therapy among patients with polymyalgia rheumatica. Arthritis Rheum. 1997;40(10):1873–8.PubMedCrossRefGoogle Scholar
  62. 62.
    Curtis JR, Westfall AO, Allison J, et al. Population-based assessment of adverse events associated with long-term glucocorticoid use. Arthritis Rheum. 2006;55(3):420–6.PubMedCrossRefGoogle Scholar
  63. 63.
    van Staa TP, Leufkens HGM, Cooper C. The epidemiology of corticosteroid-induced osteoporosis: a meta-analysis. Osteoporos Int. 2002;13(10):777–87.PubMedCrossRefGoogle Scholar
  64. 64.
    Boomsma MM, Stegeman CA, Kramer AB, Karsijns M, Piers DA, Tervaert JWC. Prevalence of reduced bone mineral density in patients with anti-neutrophil cytoplasmic antibody associated vasculitis and the role of immunosuppressive therapy: a cross-sectional study. Osteoporos Int. 2002;13(1):74–82.PubMedCrossRefGoogle Scholar
  65. 65.
    Teuscher AU, Reinli K, Teuscher A, Investigators S. Glycaemia and insulinaemia in elderly European subjects (70–75 years). Diabet Med. 2001;18(2):150–3.PubMedCrossRefGoogle Scholar
  66. 66.
    Wang JJ, Rochtchina E, Tan AG, Cumming RG, Leeder SR, Mitchell P. Use of inhaled and oral corticosteroids and the long-term risk of cataract. Ophthalmology. 2009;116(4):652–7.PubMedCrossRefGoogle Scholar
  67. 67.
    Klein BEK, Klein R, Lee KE, Dansforth LG. Drug use and five-year incidence of age-related cataracts: The Beaver Dam Eye Study. Ophthalmology. 2001;108(9):1670–4.PubMedCrossRefGoogle Scholar
  68. 68.
    de Jonghe B, Lacherade JC, Sharshar T, Outin H. Intensive care unit-acquired weakness: risk factors and prevention. Crit Care Med. 2009;37(10 Suppl):S309–15.PubMedCrossRefGoogle Scholar
  69. 69.
    Turnheim K. Drug therapy in the elderly. Exp Gerontol. 2004;39(11–12):1731–8.PubMedCrossRefGoogle Scholar
  70. 70.
    Cohen P, Pagnoux C, Mahr A, et al. Churg-Strauss syndrome with poor-prognosis factors: a prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients. Arthritis Rheum. 2007;57(4):686–93.PubMedCrossRefGoogle Scholar
  71. 71.
    Mazlumzadeh M, Hunder GG, Easley KA, et al. Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo-controlled, randomized prospective clinical trial. Arthritis Rheum. 2006;54(10):3310–8.PubMedCrossRefGoogle Scholar
  72. 72.
    McLean AJ, Le Couteur DG. Aging biology and geriatric clinical pharmacology. Pharmacol Rev. 2004;56(2):163–84.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.University of ArizonaTucsonUSA

Personalised recommendations