Abstract
Small-vessel vasculitis is a convenient descriptor for a wide range of diseases characterized by vascular inflammation of the venules, capillaries, and/or arterioles with plemorphic clinical manifestations. The classical clinical phenotype is leukocytoclastic vasculitis with palpable purpura, but manifestations vary widely depending upon the organs involved. Histopathologic examination in leukocytoclastic vasculitis reveals angiocentric segmental inflammation, fibrinoid necrosis, and a neutrophilic infiltrate around the blood vessel walls with erythrocyte extravasation. The etiology of small-vessel vasculitis is unknown in many cases, but in others, drugs, post viral syndromes, malignancy, primary vasculitis such as microscopic polyarteritis, and connective tissue disorders are associated. The diagnosis of small-vessel vasculitis relies on a thorough history and physical examination, as well as relevant antibody testing including antinuclear antibody and antineutrophil cytoplasmic antibody, hepatitis B and C serologies, assessment of complement, immunoglobulins, blood count, serum creatinine, liver function tests, urinalysis, radiographic imaging, and biopsy.
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References and Recommended Reading
Iglesias-Gamarra A: Vasculitis refractarias: aspectos generales. Rev Col Reumatol 1999, 6:144–160.
Iglesias-Gamarra A, Valle R, Egea E, et al.: Análisis histórico de las vasculitis, su clasificación y propuesta para el entendimiento. Biomédica 1993, 13:38–56.
Csernok E: Anti-neutrophil cytoplasmic antibodies and pathogenesis of small vessel vasculitides. Autoimmun Rev 2003, 2:158–164.
Davies DJ, Moran JE, Niall JF, Ryan GB: Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed) 1982, 285:606.
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, 318:1651–1657.
Jennette JC, Falk RJ: Anti-neutrophil cytoplasmic autoantibodies: discovery, specificity, disease associations and pathogenic potential. Adv Pathol Lab Med 1995, 8:363–378.
Goldschmeding R, van der Schoot CE, ten Bokkel Huinink D, et al.: Wegener’s granulomatosis autoantibodies identify a novel diisopropylfluorophosphate-binding protein in the lysosomes of normal human neutrophils. J Clin Invest 1989, 84:1577–1587.
Ludemann J, Utecht B, Gross WL: Anti-neutrophil cytoplasm antibodies in Wegener’s granulomatosis recognize an elastinolytic enzyme. J Exp Med 1990, 171:357–362.
Gross WL, Schmitt WH, Csernok E: ANCA and associated diseases: immunodiagnostic and pathogenetic aspects. Clin Exp Immunol 1993, 91:1–12.
Zeek PM: Periarteritis nodosa and other forms of necrotizing angiitis. N Engl J Med 1953, 248:764–772.
Sams HH, Sams Jr WM: Cutaneous leukocytoclastic vasculitis. In Vasculitis. Edited by Ball GV, Bridger L Jr. Oxford, UK: Oxford University Press; 2002:467–475.
Jennette JC, Falk RJ: Small-vessel vasculitis. N Engl J Med 1997, 337:1512–1523.
Cantillo Turbay J, Iglesias A, Restrepo JF: Análisis critico de las clasificaciones de las vasculitis. Rev Col Reumatol 2006, 13:48–64.
Hautmann G, Campanile G, Lotti TM: The many faces of cutaneous vasculitis. Clin Dermatol 1999, 17:515–531.
Callen JP: Cutaneous vasculitis and its relationship to systemic disease. In Inflammatory Diseases of Blood Vessel. Edited by GS Hoffman, CM Weyand. New York: Marcel Dekker, Inc.; 2002:529–538.
Iglesias-Gamarra A, Valle R, Abud Mendoza C: Vasculitis de pequeños vasos. In Tratado Hispanoamericano de Reumatología. Edited by Molina J, Alarcón Segovia D. Bogota: Editorial Nomos SA; 2007:in press.
Callen JP: Cutaneous vasculitis: relationship to systemic disease and therapy. Curr Prob Dermatol 1993, 5:45–80.
Mullick FG, McAllister HA Jr, Wagner BM, Fenoglio JJ Jr: Drug related vasculitis. Clinicopathologic correlations in 30 patients. Hum Pathol 1979, 10:313–325.
Soter NA, Mihm MC Jr, Gigli I, et al.: Two distinct cellular patterns in cutaneous necrotizing angiitis. J Invest Dermatol 1976, 66:344–350.
Soter NA: Cutaneous necrotizing venulitis. In Dermatology in General Medicine Vol. 1. Edited by Fitzpatrik TB, Eisen AZ, Wolff K. New York: McGraw Hill; 1993:1501–1510.
Crowson AN, Mihm MC Jr, Magro CM: Cutaneous vasculitis: a review. J Cutan Pathol 2003, 30:161–173.
Fiorentino DF: Cutaneous vasculitis. J Am Acad Dermatol 2003, 48:311–343.
Black AK: Urticarial vasculitis. Clin Dermatol 1999, 17:565–569.
Wisnieski JJ: Urticarial vasculitis. Curr Opin Rheumatol 2000, 12:24–31.
Schwartz HR, McDuffie FC, Black LF, et al.: Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease. Mayo Clin Proc 1982, 57:231–238.
Mehregan DR, Hall MJ, Gibson LE: Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol 1992, 26:441–448.
Wisnieski JJ, Baer AN, Christensen J, et al.: Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore) 1995, 74:24–41.
Woywodt A, Schneider W, Morack G, et al.: Necrotizing small-vessel vasculitis confined to the uterine cervix. Semin Arthritis Rheum 2000, 29:368–372.
Alexander EL, Moyer C, Travlos GS, et al.: Two histopathologic types of inflammatory vascular disease in MRL/Mp autoimmune mice, model for human vasculitis in connective tissue disease. Arthritis Rheum 1985, 28:1146–1155.
Massa MC, Su WP: Lymphocytic vasculitis: is it a specific clinicopathologic entity? J Cutan Pathol 1984, 11:132–139.
Natbony SF, Phillips ME, Elias JM, et al.: Histopathologic studies of chronic idiopathic urticaria. J Allergy Clin Immunol 1983, 71:132–139.
Egea E, Garavito de Egea G, Severino S, et al.: Vasculitis linfomonocitica informe de nueve casos. Acta Med Colomb 1987, 12:330–338.
Ghersetich I, Comacchi, Jorizzo JL, et al.: Proposal for a working classification of cutaneous vasculitis. Clin Dermatol 1999, 17:499–503.
Calvino MC, Llorca J, Garcia-Porrua C, et al.: Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 2001, 80:279–290.
Martínez-Taboada VM, Blanco R, García-Fuentes M, et al.: Clinical features and outcome of 95 patients with hyper-sensitivity vasculitis. Am J Med 1997, 102:186–191.
Chen M, Yu F, Zhang Y, et al.: Characteristics of Chinese patients with Wegener’s granulomatosis with anti-myeloperoxidase autoantibodies. Kidney Int 2005, 68:2225–2229.
Gibson A, Stamp LK, Chapman PT, et al.: The epidemiology of Wegener’s granulomatosis and microscopic polyangiitis in a Southern Hemisphere region. Rheumatology (Oxford) 2006, 45:624–628.
Lotti T, Comacchi C, Ghersetich I: Cutaneous necrotizing vasculitis. Int J Dermatol 1996, 35:457–474.
Piette WW: The differential diagnosis of purpura from a morphologic perspective. Adv Dermatol 1994, 9:3–24.
LeBoit PE: Vasculitis: the true and the near-true. Am J Dermatopathol 2002, 24:267–269.
Pillebout E, Thervet E, Hill G, et al.: Henoch-Schönlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 2002, 13:1271–1278.
Sinico RA, Di Toma L, Maggiore U, et al.: Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005, 52:2926–2935.
Tervaert JW, van der Woude FJ, Fauci AS, et al.: Association between active Wegener’s granulomatosis and anticytoplasmic antibodies. Arch Intern Med 1989, 149:2461–2465.
Tervaert JW, Huitema MG, Hene RJ, et al.: Prevention of relapses in Wegener’s granulomatosis by treatment based on antineutrophil cytoplasmic antibody titre. Lancet 1990, 336:709–711.
Hogan SL, Falk RJ, Chin H, et al.: Predictors of relapse and treatment resistance in antineutrophil, cytoplasmic, antibody-associated small-vessel vasculitis. Ann Intern Med 2005, 143:621–631.
Kyndt X, Reumaux D, Bridoux F, et al.: Serial measurements of antineutrophil cytoplasmic autoantibodies in patients with systemic vasculitis. Am J Med 1999, 106:527–533.
Koldingsnes W, Nossent JC: Baseline features and initial treatment as predictors of remission and relapse in Wegener’s granulomatosis. J Rheumatol 2003, 30:80–88.
Stegeman CA, Tervaert JW, Sluiter WJ, et al.: Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med 1994, 120:12–17.
Popa ER, Stegeman CA, Bos NA, et al.: Staphylococcal super-antigens and T cell expansion in Wegener’s granulomatosis. Clin Exp Immunol 2003, 132:496–504.
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Iglesias-Gamarra, A., Restrepo, J.F. & Matteson, E.L. Small-vessel vasculitis. Curr Rheumatol Rep 9, 304–311 (2007). https://doi.org/10.1007/s11926-007-0049-3
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DOI: https://doi.org/10.1007/s11926-007-0049-3