Scleroderma renal crisis: a rare life-threatening cause of hypertension

Abstract

Scleroderma renal crisis is a rare and severe complication of systemic sclerosis. There are many risk predictors for the development of this complication. Diagnostic criteria are non-consensual, including arterial hypertension and oliguric acute kidney injury (AKI). Angiotensin-converting enzyme inhibitors are an effective treatment, with huge impact in prognosis. We describe here the case of a new diagnosis of scleroderma renal crisis in a 78-years-old woman who was diagnosed with systemic sclerosis and treated with prednisone at high dosage, who presented with new onset congestive heart failure, arterial hypertension and oliguric AKI. The diagnosis of scleroderma renal crisis was performed, and angiotensin-converting enzyme inhibitor was initiated with blood pressure control and slightly improved renal function. The prognosis of scleroderma renal crisis remains poor with high 5-year mortality rate. Medical awareness on tensional values and well-known risk factors could have a huge impact in diagnosis and prognosis and even on prevention of this complication.