Jornal Vascular Brasileiro
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

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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.


Systemic sclerosis, digital ischemia, bosentan


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.


Esclerose sistêmica, isquemia digital, bosentan


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