Jornal Vascular Brasileiro
https://jvascbras.org/article/doi/10.1590/S1677-54492007000300011
Jornal Vascular Brasileiro
Case Report

Severe digital ischemia due to systemic sclerosis successfully treated with bosentan: case report

Tratamento de isquemia digital severa em paciente com esclerose sistêmica utilizando bosentana: relato de caso

Cesar José Guimarães Soares; Boris A. Cruz

Downloads: 0
Views: 1156

Abstract

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by cutaneous and visceral fibrosis and widespread vascular pathology. Raynaud's phenomenon is one of the most common manifestations of SSc. It may lead to complications such as digital ulceration and infarction and its treatment remains elusive. We present the case of a female patient with severe digital ischemia secondary to SSc. Treatment using vasodilators and prostacyclin analogues was ineffective, but the patient experienced a remarkable response to bosentan, a dual endothelin receptor antagonist. This report suggests that endothelin antagonists may benefit patients with SSc and vasculopathy as a major feature of the disease, including limb ischemia.

Keywords

Systemic sclerosis, digital ischemia, bosentan

Resumo

Esclerose sistêmica (SSc) é uma doença auto-imune do tecido conjuntivo caracterizada por fibrose cutânea e visceral, além de vasculopatia difusa.Ofenômeno deRaynaud é uma das manifestações mais comuns da SSc, que pode levar a complicações como úlceras digitais e infarto. Seu tratamento ainda é indefinido. Apresentamos o caso de uma paciente com isquemia digital grave secundária a SSc. O tratamento incluindo vasodilatadores e análogos de prostaciclina foi ineficaz, mas a paciente apresentou excelente resposta ao bosentan, um antagonista de receptores de endotelina. Este relato sugere que antagonistas de endotelina podem beneficiar pacientes com SSc e vasculopatia como principal característica da doença, incluindo isquemia de extremidades.

Palavras-chave

Esclerose sistêmica, isquemia digital, bosentan

References

Korn JH. Pathogenesis of systemic sclerosis. Arthritis and allied conditions. 2001:1643-54.

Block JA, Sequeira W. Raynaud's phenomenon. Lancet. 2001;357:2042-8.

Desai R, Korn JH. Diagnosis and management of Raynaud phenomenon. J Musculoskel Med.. 2003;20:124-35.

LeRoy EC, Black C, Fleischmajer R. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol.. 1988;15:202-5.

Jimebez AS, Derk CT. Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann Intern Med.. 2004;140:37-50.

Campbell PM, LeRoy EC. Pathogenesis of systemic sclerosis: a vascular hypothesis. Semin Arthritis Rheum.. 1975;4:351-68.

Gryglewski RJ, Botting RM, Vane JR. Mediators produced by the endothelial cell. Hypertension. 1988;12:530-48.

Inoue A, Yanagisawa M, Kimura S. The human endothelin family: three structurally and pharmacologically distinct isopeptides predicted by three separate genes. Proc Natl Acad Sci USA.. 1989;86:2863-7.

Biondi ML, Marasini B, Bassani C, Agostoni A. Increased plasma endothelin levels in patients with Raynaud's phenomenon. N Engl J Med.. 1991;324:1139-40.

Rubin LJ, Badesch DB, Barst RJ. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med.. 2002;346:896-903.

Sitbon O, Badesch DB, Channick RN. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest. 2003;124:247-54.

Korn JH, Mayes M, Matucci Cerinic M. Digital ulcers in systemic sclerosis: Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum.. 2004;50:3985-93.

Seibold JR, Matucci-Cerinic M, Denton CP. Bosentan reduces the number of new digital ulcers in patients with systemic sclerosis. Ann Rheum Dis.. 2006;65(^sII):S90.

Sociedade Brasileira de Angiologia e Cirurgia Vascular (SBACV)"> Sociedade Brasileira de Angiologia e Cirurgia Vascular (SBACV)">
5ddec0b00e88259c157279a1 jvb Articles
Links & Downloads

J Vasc Bras

Share this page
Page Sections