Síndrome de Churg-Strauss

Autores

  • B. F. Rodrigues Serviço de Nefrologia e Serviço de Medicina I do Hospital Fernando Fonseca
  • J. M. Araújo Serviço de Nefrologia e Serviço de Medicina I do Hospital Fernando Fonseca

Palavras-chave:

síndrome de Churg-Strauss, ANCA, vasculite

Resumo

A síndrome de Churg-Strauss é uma vasculite ANCA-associada de
pequenos e médios vasos. As manifestações clínicas mais comuns
são eosinofilia marcada, asma, sinusite crónica, infiltrados pulmonares, cardiomiopatia, queixas gastrointestinais e mononeurite
multiplex. Os anticorpos Anti-MPO (anti-mieloperoxidade), pANCA
(com padrão de imunoflurescência perinuclear) encontram-se
presentes em 38-59% dos casos. O envolvimento cardíaco é
uma importante causa de morbilidade e a principal causa de
mortalidade no síndrome de Churg-Strauss. Histologicamente
caracteriza-se por uma vasculite necrotizante eosinofílica.
O tratamento é baseado na corticoterapia e em fármacos imunosupressores (ciclofosfamida e azatioprina) sendo determinado
de acordo com critérios de prognóstico.
A remissão completa ocorre em cerca de 90% dos casos,
sendo as recaídas frequentes (25% dos casos).

Downloads

Não há dados estatísticos.

Referências

Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrouse B, Cassaus P. Churg-Strauss Syndrome: Clinical study and long term follow up of 96 patients. Medicine 1999; 78:26-37.

Abril A, et al. The Churg Strauss Syndrome (Allergic Granulomatous Angiitis): Review and Update. Seminars in Arthritis and Reumatism 2003; 33 (2):106-114.

Finan MC, Winkelmann RK. The cutaneous extravascular necrotizing granuloma (Churg-Strauss granuloma) and systemic disease: a review of 27 cases. Medicine (Baltimore). 1983;62(3):142-158.

Harrold LR, Andrade SE, Go AS, Buist AS, Eisner M, Vollmer WM, Chan KA, Frazier EA, Weller PF, Wechsler ME, Yood RA, Davis KJ, Platt R. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective. J Rheumatol. 2005;32(6):1076-1080.

Watts RA, Curruthers DM, Scott DGI. Epidemiology of systemic vasculitis: Changing incidence or definition? Semin Arthritis Rheum 1995;25:28-34.

Kurland LT, Chuang TY, Hunder GG: The epidemiology of systemic arteritis, in Lawrence RC, Shulman LE, (eds): The epidemiology of the Rheumatic Diseases. New York, Gower 1984:196-205.

Martin RM, Wilton LV, Mann RD. Prevalence of Churg-Strauss syndrome, vasculitis, eosinophilia and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies. Pharmacoepidemiol Drug Saf. 1999;8(3):179-189.

Haugeberg G, Bie R, Bendvold A, Larsen AS, Johnsen V.; Primary vasculitis in a Norwegian community hospital: a retrospective study. Clin Rheumatol. 1998;17(5):364-368.

Franco J, Artés MJ. Pulmonary eosinophilia associated with montelukast. Thorax. 1999;54(6):558-560.

Wechsler ME, Garpestad E, Flier SR, Kocher O, Weiland DA, Polito AJ, Klinek MM, Bigby TD, Wong GA, Helmers RA, Drazen JM. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. 1998;279(6):455-457.

Wechsler ME, Finn D, Gunawardena D, Westlake R, Barker A, Haranath SP, Pauwels RA, Kips JC, Drazen JM. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. 2000;117(3):708-713.

Weller PF, Plaut M, Taggart V, Trontell A. The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report. J Allergy Clin Immunol. 2001;108(2):175-183.

Chen KR, Ohata Y, Sakurai M, Nakayama H. Churg-Strauss syndrome: report of a case without preexisting asthma. J Dermatol. 1992 ;19(1):40-47.

Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet. 2003 ;361(9357):587-594.

Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187-192.

Churg J, Strauss L. Allergic Granulomatosis, Allergic Angiitis, and Periarteritis Nodosa Am J Pathol. 1951;27(2):277-301.

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.

Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY et al. The American College of

Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33(8):1094-1100.

Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc. 1977;52(8):477-484.

Moore PM, Calabrese LH. Neurologic manifestations of systemic vasculitides. Semin Neurol. 1994;14(4):300-306.

Namora J., Santos FM, Morais T et al. Síndrome de Churg-Strauss – Caso Clínico. Acta Reuma Port. 2000; 98:19-23.

Oliveira S., Mendonça C., Ambar J., Mendes B. Síndroma de Churg-Strauss – a propósito de um caso clínico. Rev Port Pneumol. 2005; XI (1): 73-83.

Hattori N, Ichimura M, Nagamatsu M, Li M, Yamamoto K, Kumazawa K, Mitsuma T, Sobue G. Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. Brain. 1999;122 ( Pt 3):427-439.

Davis MD, Daoud MS, McEvoy MT, Su WP. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997;37(2 Pt 1):199-203.

Lie JT. Limited forms of Churg-Strauss syndrome. Pathol Annu. 1993;28 Pt 2:199-220.

Clutterbuck EJ, Evans DJ, Pusey CD. Renal involvement in Churg-Strauss syndrome. Nephrol Dial Transplant. 1990;5(3):161-167.

Reid AJ, Harrison BD, Watts RA, Watkin SW, McCann BG, Scott DG. Churg-Strauss syndrome in a district hospital. QJM. 1998;91(3):219-229.

Solans R, Bosch JA, Pérez-Bocanegra C, Selva A, Huguet P, Alijotas J, Orriols R, Armadans L, Vilardell M. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford). 2001;40(7):763-771.

Schmitt WH, Csernok E, Kobayashi S. Churg Strauss syndrome serum markers of lymphocyte activation and endothelial damage. Arthritis Rheum 1998; 41:445.

Hasley PB, Follansbee, WP, Coulehan, JL. Cardiac manifestations of Churg-Strauss syndrome: Report of a case and review of the literature. Am Heart J 1990; 120:996.

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.

Lhothe F, Cohen P, Guillevin L. Syndrome de Churg et Strauss. Maladies et syndromes systémiques. 5e édition. Médecine-Sciences, Flammarion. Paris, 2008; 652-673.

Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am, 1995; 21:883-910.

Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997 Nov 20;337(21):1512-23.

Silva M, Garrido N, Alves P et al. Efeitos diferenciais da terapêutica córtico-imunossupressora nas diversas manifestações da síndrome de Churg-Strauss: a propósito de um caso clínico. Med Interna, 2005; 12(3): 154-158.

Lanham JG. Churg-Strauss syndrome. Br J Hosp Med. 1992;47(9):667-673.

Groot, K., Adu, D. The value of cyclophosphamide in ANCA-associated vasculitis: Meta-analysis and critical review. Nephrol. Dial. Transplan. 2001;16: 2018-2027.

Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R; European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis.

;68(3):310-317.

Langford CA, Talar-Williams C, Sneller MC. Mycophenolate mofetil for remission maintenance in the treatment of Wegener’s granulomatosis. Arthritis Rheum. 2004 15;51(2):278-283.

De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, Gross WL, Luqmani R, Jayne DR. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(8):2237-2242.

Metzler C, Fink C, Lamprecht P, Gross WL, Reinhold-Keller E. Maintenance of remission with leflunomide in Wegener’s granulomatosis. Rheumatology (Oxford). 2004;43(3):315-320.

Guillevin L, Cevallos R, Durand-Gasselin B et al. Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis. Ann Med Interne (Paris) 1997;148(3):198-204.

Metzler C, Schnabel A, Gross WL, Hellmich B. A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome. Clin Exp Rheumatol. 2008;26(3 Suppl 49):S35-40.

Jones RB, Ferraro AJ, Chaudhry AN et al. A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasm antibody-associated vasculitis. Arthritis Rheum. 2009 ;60(7):2156-2168

Ficheiros Adicionais

Publicado

30-09-2011

Como Citar

1.
Rodrigues BF, Araújo JM. Síndrome de Churg-Strauss. RPMI [Internet]. 30 de Setembro de 2011 [citado 16 de Abril de 2024];18(3):166-72. Disponível em: https://revista.spmi.pt/index.php/rpmi/article/view/1330

Edição

Secção

Artigos de Revisão