Abstract
Vasculitides in the setting of connective tissue diseases are generally thought to be infrequent and relatively little is written about them. They are, however, important both because they may pose diagnostic and therapeutic challenges and affect prognosis. In each of the connective tissue diseases, vasculitis can present in various clinical and pathologic forms adding to their diagnostic and therapeutic difficulties. This article reviews recent information on the frequency, characteristics, and possible pathogenic mechanisms of the vasculitides occurring in patients with the main connective tissue diseases.
Similar content being viewed by others
References and Recommended Reading
Gladman DD, Urowitz MB: Systemic lupus erythematosus: clinical features. In Rheumatology. Edited by Klippel JH, Dieppe PA. London: Mosby; 1998 (Section 7.1):1–18.
Drenkard C, Villa AR, Reyes E, et al.:Vasculitis in systemic lupus erythematosus. Lupus 1997, 6:235–242. This is the largest and most detailed study of vasculitis in systemic lupus erythematosus. An association of vasculitis with antiphospholipid antibodies and the antiphospholipid syndrome is well demonstrated.
Carreño L, López-Longo FJ, Monteagudo I, et al.:Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus 1999, 8:287–292.
Alarcón-Segovia D, Alarcón-Riquelme ME: Etiopathogenesis of systemic lupus erythematosus: a tale of three troikas. In Systemic Lupus Erythematosus. Edited by Lahita RG. San Diego: Academic Press; 1999:55–65.
Shoenfeld Y, Alarcón-Segovia D, Buskila D, et al.:Frontiers of SLE: review of the 5th International Congress of systemic lupus erythematosus, Cancún, México, April 20–25, 1998. Semin Arthritis Rheum 1999, 29:112–130.
Carvalho D, Savage COS, Isenberg D, Pearson JD: IgG antiendothelial cell autoantibodies from patients with systemic lupus erythematosus stimulate the release of two endothelial cell-derived mediators, which enhance adhesion molecule expression and leukocyte adhesion in an autocrine manner. Arthritis Rheum 1999, 42:631–640.
Reyes E, Alarcón-Segovia D: Leg ulcers in the primary antiphospholipid syndrome: report of a case with a peculiar proliferative small vessel vasculopathy. Clin Exp Rheumatol 1991, 9:63–66.
Del Papa N, Sheng YH, Raschi E, et al.:Human b2-glycoprotein I binds to endothelial cells through a cluster of lysine residues that are critical for anionic phospholipid binding and offers epitopes for anti b2-glycoprotein I antibodies. J Immunol 1998, 160:5572–5578. A good demonstration of the interplay of several actors in the pathogenesis of the antiphospholipid syndrome that may help understand its association with vasculitis.
Villarreal GM, Drenkard C, Villa AR, et al.:Prevalence of 13 autoantibodies and of the 16/6 and related pathogenic idiotypes in 465 patients with systemic lupus erythematosus and their relationship with disease activity. Lupus 1997, 6:425–435.
Chan OWT, Madaio MP, Shlomchik MJ: B cells are required for lupus nephritis in the polygenic, Fas-intact MRL model of systemic autoimmunity. J Immunol 1999, 163:3592–3596. This study demonstrates the requirement for B cells in the development of vasculitis in an animal model of systemic lupus erythematosus with resultant diminution of CD4 and CD8 positive T cells.
Singer GG, Carrera AC, Marshak-Rothstein A, et al.:Apoptosis, Fas and systemic autoimmunity: the MRL-lpr/lpr model. Curr Opin Immunol 1994, 6:913–920.
Chan OT, Hannum LG, Haberman AM, et al.:A novel mouse with B cells but lacking serum antibody reveals an antibodyindependent role for B cells in murine lupus. J Exp Med 1999, 189:1639–1647. B cells that are incapable of secreting autoantibodies still participate in the development of vasculitis in a murine lupus model.
Luzina IG, Knitzer RH, Atamas SP, et al.: Vasculitis in the Palmerston North mouse model of lupus: phenotype and cytokine production profile of infiltrating cells. Arthritis Rheum 1999,
Llorente L, Zou W, Levy Y, et al.:Role of interleukin-10 in the B lymphocyte hyperactivity and autoantibody production of human systemic lupus erythematosus. J Exp Med 1995, 181:839–844.
Witte T, Hartung K, Sachse C, et al.:Thrombomodulin in systemic lupus erythematosus: association with clinical and laboratory parameters. Rheumatol Int 1999, 19:15–18.
Bacon PA, Carruthers DM: Vasculitis associated with connective tissue disorders. Rheum Dis Clin North Am 1995, 21:1077–1096.
Sundy JS, Haynes BF: Rheumatoid arthritis. In The Autoimmune Diseases. Edited by Rose NR, Mackay IR. San Diego: Academic Press; 1998:343–380.
Bacon PA, Kitas GD: The significance of vascular inflammation in rheumatoid arthritis. Ann Rheum Dis 1994, 53:621–623.
Schwarz MI, Zamora MR, Hodges TN, et al.:Isolated pulmonary capillaritis and diffuse alveolar hemorrhage in rheumatoid arthritis and mixed connective tissue disease. Chest 1998, 113:1609–1615.
Turesson C, Jacobson L, Bergström U: Extra-articular rheumatoid arthritis: prevalence and mortality. Rheumatology 1999, 38:668–674.
Kuryliszyn-Moskal A: Cytokines and soluble CD4 and CD8 molecules in rheumatoid arthritis: relationship to systematic vasculitis and microvascular capillaroscopic abnormalities. Clin Rheumatol 1998, 17:489–495.
Prigione I, Buoncompagni A, Cutolo M, et al.:Normal levels of soluble CD4 in sera from patients with juvenile chronic arthritis. Clin Exp Rheumatol 1993, 11:341–344.
Blann AD, Herrick A, Jayson MIV: Altered levels of soluble adhesion molecules in rheumatoid arthritis, vasculitis and systemic sclerosis. Br J Rheumatol 1995, 34:814–819.
Salih AM, Nixon NA, Dawes PT, Mattey DL: Soluble adhesion molecules and anti-endothelial cell antibodies in patients with rheumatoid arthritis complicated by peripheral neuropathy. J Rheumatol 1999, 26:551–555.
Alarcón-Segovia D, Cardiel MH, Reyes E: Antiphospholipid arterial vasculopathy. J Rheumatol 1989, 16:762–767.
Gibson GE, Su WPD, Pittelkow MR: Antiphospholipid syndrome and the skin. J Am Acad Dermatol 1997, 36:970–982.
Norden DK, Ostrov BE, Shafritz AB, von Feldt JM: Vasculitis associated with antiphospholipid syndrome. Semin Arthritis Rheum 1995, 24:273–281.
Goldberger E, Elder RC, Schwartz RA, Phillips PE: Vasculitis in the antiphospholipid syndrome. Arthritis Rheum 1992, 35:569–572.
Cervera R, Khamashta MA, Font J, et al.:Antiendothelial cell antibodies in patients with the antiphospholipid syndrome. Autoimmunity 1991, 11:1–6.
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.
Tzioufas AG, Moutsopoulos HM: Sjögren’s syndrome. In Rheumatology. Edited by Klippel JH, Dieppe PA. London: Mosby; 1998:1–12.
Praprotnik S, Bozic B, Kveder T, Rozman B: Fluctuation of anti-Ro/SS-A antibody levels in patients with systemic lupus erythematosus and Sjögren’s syndrome: a prospective study. Clin Exp Rheumatol 1999, 17:63–68.
Alexander EL, Provost TT: Cutaneous manifestations of primary Sjögren’s syndrome: a reflection of vasculitis and association with anti-Ro (SS-A) antibodies. J Invest Dermatol 1983, 80:386–391.
Niemela RK, Hakala M: Primary Sjögren’s syndrome with severe central nervous system disease. Semin Arthritis Rheum 1999, 29:4–13.
Huerta-Yañez G, Sánchez-Guerrero J, Reyes E, Alarcón-Segovia D: Vasculitis en enfermedad mixta del tejido conjuntivo (EMTC) [abstract]. Rev Mex Reumatol 1989, 4:3.
Kraus A, Cervantes G, Barojas E, Alarcón-Segovia D: Retinal vasculitis in mixed connective tissue disease. A fluoroangiographic study. J Rheumatol 1985, 12:1122–1124.
Ishikawa O, Tamura T, Ohnishi K, et al.:Systemic sclerosis terminating as systemic necrotizing angiitis. Br J Dermatol 1993, 129:736–738.
Herrick AL, Oogarah PK, Freemont AJ, et al.:Vasculitis in patients with systemic sclerosis and severe digital ischaemia requiring amputation. Ann Rheum Dis 1994, 53:323–326.
Oddis CV, Eisenbeis CH Jr, Reidbord HE, et al.:Vasculitis in systemic sclerosis: association with Sjögren’s syndrome and the CREST syndrome variant. J Rheumatol 1987, 14:942–948.
Dyck PJB, Hunder GG, Dyck PJ: A case-control and nerve biopsy study of CREST multiple mononeuropathy. Neurology 1997, 49:1641–1645. Of a large group of patients with CREST syndrome, the authors study in detail those who had neuropathy, mostly related to vasculitis.
Banker BQ, Victor M: Dermatomyositis (systemic angiopathy) in childhood. Medicine 1966, 45:261–289.
Koh ET, Seow A, Ong B, et al.:Adult onset polymyositis/ dermatomyositis: clinical and laboratory features and treatment response in 75 patients. Ann Rheum Dis 1993, 52:857–861.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Flores-Suárez, L.F., Alarcón-Segovia, D. Vasculitis in the connective tissue diseases. Curr Rheumatol Rep 2, 396–401 (2000). https://doi.org/10.1007/s11926-000-0039-1
Issue Date:
DOI: https://doi.org/10.1007/s11926-000-0039-1