Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature

Abstract

Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) that is characterised by new-onset malignant hypertension and progressive acute renal failure, often with associated microangiopathic haemolytic anaemia and thrombocytopenia. SRC was at one time almost uniformly fatal, with death often occurring within a few weeks. With the development of angiotensin-converting-enzyme inhibitors (ACE-I), survival has improved dramatically, but death rates still remain unacceptably high. About 20% of SRC cases occur prior to making a diagnosis of SSc and, in some cases, there is no evidence of skin sclerosis at the time that SRC develops. In this report, we present a case in which a patient developed SRC prior to being diagnosed with scleroderma. Additionally, we review the pathogenesis, presenting signs and symptoms, management and prognosis of SRC.