Progressive systemic sclerosis - An unusual presentation

• Autor(a) principal: Ribeiro da Cunha, Ana
• Data de Publicação: 1996
• Outros Autores: Lourenço, José, Santos, Teresinha, Barros Veloso, A.
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://revista.spmi.pt/index.php/rpmi/article/view/2274
• topic: crise renal de esclerodermia; esclerose sistémica progressiva; hipertensão ar­terial secundária; scleroderma renal crisis; secondary hypertension; progressive systemic sclorosis

The authors report a case of a 28 year old wo­man, admited to the Hospital by malignant hyper­tension, retinopathy grade IV and intracranial hypertension, associated with poliarthralgia, Raynauds phenomenon, microstomy, scleroda­ ctyly and acute renal failure. After complete evaluation, we have excluded other causes of secondary hypertension and di­ agnosed progressive systemic sclerosis with mul­tisystem envolvement (kidney, skin, lung, esopha­gus and retine), that had a rare form of presen­tation scleroderma renal crisis (10% of cases) and started emergent antihypertensive treatment with ACE inhibitors (captopril 150 mg daily), ni­fedipine (60 mg daily) and dialysis. We emphazi­se this clinical case because it s a rare disease (2.7 new patients/million/year) that had a rare form of presentation, and had a complete reco­very of renal function after three months of he­ modialysis. She is at the present date with nor­ mal blood pressure without any treatment.