To discuss the pathophysiology, risk factors, clinical manifestations, diagnosis, treatment, prevention, and outcomes of scleroderma renal crisis (SRC), a serious yet potentially treatable complication of scleroderma (systemic sclerosis).
A PubMed search for articles published up until April 2014 was conducted using the following keywords: scleroderma, systemic sclerosis, scleroderma renal crisis, renal, treatment, and prognosis. Literature was carefully reviewed, and different risk factors, treatment options, prognostic factors, and survival data were assessed.
SRC occurs in about 10% of all patients with scleroderma. It is characterized by malignant hypertension and progressive renal failure. Around 10% of SRC cases may present with normal blood pressure, termed normotensive renal crisis. The etiopathogenesis is presumed to be a series of insults to the kidneys resulting in endothelial injury, intimal proliferation, and narrowing of renal arterioles leading to decreased blood flow, hyperplasia of the juxtaglomerular apparatus, hyperreninemia, and accelerated hypertension. Risk factors include rapid skin thickening, use of certain medications such corticosteroids or cyclosporine, new-onset microangiopathic hemolytic anemia and/or thrombocytopenia, cardiac complications (pericardial effusion, congestive heart failure, and/or arrhythmias), large joint contractures, and presence of anti-RNA polymerase III antibody. Since the 1970s, with the advent of angiotensin-converting enzyme (ACE) inhibitors, mortality associated with SRC decreased from 76% to <10%. Some patients may progress to end-stage renal disease and need dialysis. Renal transplantation has improved survival, though SRC may recur in transplanted kidneys.
More than 60 years after its initial description, SRC still remains an important cause of morbidity and mortality in scleroderma. Since the advent of ACE inhibitors, the prognosis of SRC has improved substantially. Prompt diagnosis and treatment may help prevent adverse outcomes and improve survival.