Scleroderma renal crisis: new insights and developments.

Scleroderma renal crisis (SRC) was once a uniformly fatal complication of systemic sclerosis (SSc). With the introduction of angiotensin-converting enzyme inhibitors as treatment, outcomes have improved significantly, though 39% to 50% of SSc patients who develop SRC continue to have poor outcomes, including permanent dialysis and death. Early recognition and treatment with angiotensin-converting enzyme inhibitors are important in the effective management of SRC, though given the continuing morbidity and mortality caused by SRC, they are clearly not sufficient. Newer therapies based on the pathophysiologic mechanisms underlying the development and perpetuation of SRC are needed. This article reviews the epidemiology, pathogenesis, risk factors, clinical features, and treatment of SRC, with an emphasis on recent insights and developments.