Skip to main content

Advertisement

SpringerLink
Log in

Vasculitis associated with connective tissue disorders

  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Vasculitis associated with connective tissue disorders is an important cause of secondary vasculitis about which little is written. When vasculitis occurs in the setting of a preexisting connective tissue disorder, it often correlates with disease severity and portends a poorer prognosis. It may involve virtually any organ system and present in a myriad of ways. Prompt recognition and treatment of vasculitis can dramatically improve the outcome for the patient. This review focuses on recent insights into the clinical presentation and pathogenic mechanisms that may be involved.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Sneller MC, Fauci AS: Pathogenesis of vasculitis syndromes. Med Clin North Am 1997, 81:221–242.

    Article  PubMed  CAS  Google Scholar 

  2. Hunder GG, Arend WP, Bloch DA, et al.: The American College of Rheumatology 1990 criteria for the classification of vasculitis. Arthritis Rheum 1990, 33:1065–1067.

    Article  PubMed  CAS  Google Scholar 

  3. Jennette JC, Falk RJ: Small-vessel vasculitis. N Engl J Med 1997, 337:1512–1523.

    Article  PubMed  CAS  Google Scholar 

  4. Gabriel SE, Crowson CS, Kremers HM, et al.: Survival in rheumatoid arthritis: a population-based analysis of trends over 40 years. Arthritis Rheum 2003, 48:54–58.

    Article  PubMed  Google Scholar 

  5. Voskuyl AE, Zwinderman AH, Westedt ML, et al.: Factors associated with the development of vasculitis in rheumatoid arthritis: results of a case-control study. Ann Rheum Dis 1996, 55:190–192.

    PubMed  CAS  Google Scholar 

  6. Voskuyl AE, Hazes JM, Zwinderman AH, et al.: Diagnostic strategy for the assessment of rheumatoid vasculitis. Ann Rheum Dis 2003, 62:407–413.

    Article  PubMed  CAS  Google Scholar 

  7. Suzuki A, Ohosone Y, Obana M, et al.: Cause of death in 81 autopsied patients with rheumatoid arthritis. J Rheumatol 1994, 21:33–36.

    PubMed  CAS  Google Scholar 

  8. Liang KP, Liang KV, Matteson EL, et al.: Incidence of noncardiac vascular disease in rheumatoid arthritis and relationship to extraarticular disease manifestations. Arthritis Rheum 2006, 54:642–648.

    Article  PubMed  Google Scholar 

  9. Maradit-Kremers H, Nicola PJ, Crowson CS, et al.: Cardiovascular death in rheumatoid arthritis: a population-based study. Arthritis Rheum 2005, 52:722–732.

    Article  PubMed  Google Scholar 

  10. Wallberg-Jonsson S, Cvetkovic JT, Sundqvist KG, et al.:Activation of the immune system and inflammatory activity in relation to markers of atherothrombotic disease and atherosclerosis in rheumatoid arthritis. J Rheumatol 2002, 29:875–882.

    PubMed  CAS  Google Scholar 

  11. Watts RA, Mooney J, Lane SE, Scott DG: Rheumatoid vasculitis: becoming extinct? Rheumatology 2004, 43:920–923.

    Article  PubMed  CAS  Google Scholar 

  12. Ward MM: Decreases in rates of hospitalizations for manifestations of severe rheumatoid arthritis, 1983–2001. Arthritis Rheum 2004, 50:1122–1131.

    Article  PubMed  Google Scholar 

  13. Turesson C, O’Fallon WM, Crowson CS, et al.: Occurrence of extraarticular disease manifestations is associated with excess mortality in a community based cohort of patients with rheumatoid arthritis. J Rheumatol 2002, 29:62–67.

    PubMed  Google Scholar 

  14. Turesson C, McClelland RL, Christianson TJ, Matteson EL: No decrease over time in the incidence of vasculitis or other extraarticular manifestations in rheumatoid arthritis: results from a community-based study. Arthritis Rheum 2004, 50:3729–3731.

    Article  PubMed  Google Scholar 

  15. Jarrett SJ, Cunnane G, Conaghan PG, et al.: Anti-tumor necrosis factor-alpha therapy-induced vasculitis: case series. J Rheumatol 2003, 30:2287–2291.

    PubMed  Google Scholar 

  16. van der Bijl AE, Allaart CF, Van Vugt J, et al.: Rheumatoid vasculitis treated with infliximab. J Rheumatol 2005, 32:1607–1609.

    PubMed  Google Scholar 

  17. Langford CA, Sneller MC: Biologic therapies in the vasculitides. Curr Opin Rheumatol 2003, 15:3–10.

    Article  PubMed  Google Scholar 

  18. Belizna CC, Duijvestijn A, Hamidou M, Cohen Tervaert JW: Antiendothelial cell antibodies in vasculitis and connective tissue disease. Ann Rheum Dis 2006 [Epub ahead of print].

  19. van der Zee JM, Heurkens AH, van der Voort EA, et al.: Characterization of anti-endothelial antibodies in patients with rheumatoid arthritis complicated by vasculitis. Clin Exp Rheumatol 1991, 9:589–594.

    PubMed  Google Scholar 

  20. Heurkens AH, Hiemstra PS, Lafeber GJ, et al.: Anti-endothelial cell antibodies in patients with rheumatoid arthritis complicated by vasculitis. Clin Exp Immunol 1989, 78:7–12.

    PubMed  CAS  Google Scholar 

  21. Gorman JD, David-Vaudey E, Pai M, et al.: Particular HLA-DRB1 shared epitope genotypes are strongly associated with rheumatoid vasculitis. Arthritis Rheum 2004, 50:3476–3484.

    Article  PubMed  CAS  Google Scholar 

  22. Turesson C, Weyand CM, Matteson EL: Genetics of rheumatoid arthritis: Is there a pattern predicting extraarticular manifestations? Arthritis Rheum 2004, 51:853–863.

    Article  PubMed  CAS  Google Scholar 

  23. Weening JJ, D’Agati VD, Schwartz MM, et al.: The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 2004, 65:521–530.

    Article  PubMed  Google Scholar 

  24. Descombes E, Droz D, Drouet L, et al.: Renal vascular lesions in lupus nephritis. Medicine 1997, 76:355–368.

    Article  PubMed  CAS  Google Scholar 

  25. Kapoulas S, Liakos S, Karkavelas G, et al.: Thrombotic thrombocytopenic purpura associated with rapidly progressive lupus nephritis: report of two cases. Clin Nephrol 2005, 63:297–301.

    PubMed  CAS  Google Scholar 

  26. Drenkard C, Villa AR, Reyes E, et al.: Vasculitis in systemic lupus erythematosus. Lupus 1997, 6:235–242.

    PubMed  CAS  Google Scholar 

  27. Cuellar ML, Espinoza LR: Laboratory testing in the evaluation and diagnosis of vasculitis. Curr Rheumatol Rep 2000, 2:417–422.

    PubMed  CAS  Google Scholar 

  28. Renaudineau Y, Dugue C, Dueymes M, Youinou P: Antiendothelial cell antibodies in systemic lupus erythematosus. Autoimmun Rev 2002, 1:365–372.

    Article  PubMed  CAS  Google Scholar 

  29. Golan TK: Lupus vasculitis: differential diagnosis with antiphospholipid syndrome. Curr Rheumatol Rep 2002, 4:18–24.

    Article  PubMed  Google Scholar 

  30. Toubi E, Kessel A, Bamberger E, Golan TD: Systemic lupus erythematosus vasculitis: a current therapeutic overview. Curr Treat Options Cardiovasc Med 2004, 6:87–97.

    PubMed  Google Scholar 

  31. Roman MJ, Shanker BA, Davis A, et al.: Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus. N Engl J Med 2003, 349:2399–2406.

    Article  PubMed  CAS  Google Scholar 

  32. Bacon PA: Endothelial cell dysfunction in systemic vasculitis: new developments and therapeutic prospects. Curr Opin Rheumatol 2005, 17:49–55. An excellent summary on the role that endothelial dysfunction plays in systemic vasculitis and its possible link to cardiovascular disease.

    Article  PubMed  CAS  Google Scholar 

  33. Ramos-Casals M, Anaya JM, Garcia-Carrasco M, et al.:Cutaneous vasculitis in primary Sjogren syndrome: classi fication and clinical significance of 52 patients. Medicine 2004, 83:96–106. This is a comprehensive review on cutaneous vasculitis in patients with primary Sjögren’s syndrome with biopsy correlation and discussion of clinical relevance to the presence of cryoglobulins and increased mortality.

    Article  PubMed  Google Scholar 

  34. Delalande S, de Seze J, Fauchais AL, et al.: Neurologic manifestations in primary Sjogren syndrome: a study of 82 patients. Medicine 2004, 83:280–291.

    Article  PubMed  Google Scholar 

  35. Mellgren SI, Conn DL, Stevens JC, Dyck PJ: Peripheral neuropathy in primary Sjogren’s syndrome. Neurology 1989, 39:390–394.

    Article  PubMed  CAS  Google Scholar 

  36. Turkcapar N, Sak SD, Saatci M, et al.: Vasculitis and expression of vascular cell adhesion molecule-1, intercellular adhesion molecule-1, and E-selectin in salivary glands of patients with Sjogren’s syndrome. J Rheumatol 2005, 32:1063–1070.

    PubMed  CAS  Google Scholar 

  37. Cossio M, Menon Y, Wilson W, deBoisblanc BP: Lifethreatening complications of systemic sclerosis. Crit Care Clin 2002, 18:819–839.

    Article  PubMed  Google Scholar 

  38. Maes B, Van Miegham A, Messiaen T, et al.: 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.

    PubMed  CAS  Google Scholar 

  39. Dyck PJ, Hunder GG, Dyck PJ: A case-control and nerve biopsy study of CREST multiple mononeuropathy. Neurology 1997, 49:1641–1645.

    PubMed  CAS  Google Scholar 

  40. Rho YH, Choi SJ, Lee YH, et al.: Scleroderma associated with ANCA-associated vasculitis. Rheumatol Int 2006, 26:369–375. Case series of 50 cases of scleroderma associated with ANCAassociatied vasculitis, with survival analysis and clinical factors associated with the development of ANCA-associated vasculitis in these patients.

    Article  PubMed  Google Scholar 

  41. Yurdakul S, Hamuryudan V, Yazici H: Behcet syndrome. Curr Opin Rheumatol 2004, 16:38–42.

    Article  PubMed  Google Scholar 

  42. Calamia KT, Schirmer M, Melikoglu M: Major vessel involvement in Behcet disease. Curr Opin Rheumatol 2005, 17:1–8.

    Article  PubMed  Google Scholar 

  43. Letko E, Zafirakis P, Baltatzis S, et al.: Relapsing polychondritis: a clinical review. Semin Arthritis Rheum 2002, 31:384–395.

    Article  PubMed  Google Scholar 

  44. Selim AG, Fulford LG, Mohiaddin RH, Sheppard MN:Active aortitis in relapsing polychondritis. J Clin Pathol 2001, 54:890–892.

    PubMed  CAS  Google Scholar 

  45. Huerta-Yanez G: Vasculitis en enfermedad mixta del tejido conjuntivo (EMTC) [abstract; in Spanish]. Rev Mex Reumatol 1985, 12:1122–1124.

    Google Scholar 

  46. Oh YB, Jun JB, Kim CK: Mixed connective tissue disease associated with skin defects of livedoid vasculitis. Clin Rheumatol 2000, 19:381–384.

    Article  PubMed  CAS  Google Scholar 

  47. Geboes K, Dalle I: Vasculitis and the gastrointestinal tract. Acta Gastroenterol Belg 2002, 65:204–212.

    PubMed  CAS  Google Scholar 

  48. Feldman D, Hochberg MC, Zizic TM, Stevens MB: Cutaneous vasculitis in adult polymyositis/dermatomyositis. J Rheumatol 1983, 10:85–89.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Centocor Research & Development, 200 Great Valley Parkway, 19355, Malvern, PA, USA

    Mittie K. Doyle MD

Authors
  1. Mittie K. Doyle MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mittie K. Doyle MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Doyle, M.K. Vasculitis associated with connective tissue disorders. Curr Rheumatol Rep 8, 312–316 (2006). https://doi.org/10.1007/s11926-006-0015-5

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-006-0015-5

Keywords

Search

Navigation