Clinical features and prognosis of patients with scleroderma renal crisis

Background and objective: Scleroderma renal crisis is a severe complication of systemic sclerosis, which causes arterial hypertension and acute renal failure. Early treatment of these patients with ACE inhibitors may improve prognosis. We aimed to analyze the frequency, clinical and epidemiological characteristics, morbidity and mortality and prognosis of scleroderma renal crisis. Patients and methods: Retrospective study of a cohort of 328 patients with SSc, of whom 194 had the limited form, 64 the diffuse form, 49 the sine scleroderma and 21 preescleroderma. We considered different subtypes of disease: limited (188), diffuse (63), scleroderma sine scleroderma (46) and preescleroderma (21). The data were obtained from a review of medical records. The differences in the prevalence of variables were analyzed by the Fisher's test. Results: A renal crisis was observed in 14 patients (4.26%), 3 men and 11 women, 64% had the diffuse form of the disease, 28% had the limited form, and 7.69% had the scleroderma sine scleroderma. The average time was 48 months. All received ACE inhibitors. The mortality was 85% (18 months) and 85% required dialysis. Conclusions: Renal crisis is a rare manifestation of scleroderma which mainly affects patients with diffuse involvement of the disease in the early stages. These patients have a poor prognosis despite treatment with ACE inhibitors. © 2010 Elsevier Españ a, S.L. Todos los derechos reservados.