Renal dysfunction associated with vasculopathy is a common pathology in systemic sclerosis (SSc), and usually exhibits a benign course
Scleroderma renal crisis (SRC) is rare — it affects 5–15% of patients, according to studies published in the past 20 years; however, a 2015 case series suggests that the incidence of SRC has decreased to 2.4%
Predictive factors for SRC include anti-RNA polymerase III antibodies, diffuse cutaneous disease, tendon friction rubs, and arthritis; glucocorticoid treatment is a risk factor for SRC
SRC should be differentiated from ANCA-positive, rapidly progressive glomerulonephritis, as treatment regimens and patient management are different
Control of SRC-associated hypertension with angiotensin-converting-enzyme (ACE) inhibitors in patients with SSc improves outcomes; however, this treatment does not prevent SRC, and might increase SRC-associated mortality
Although prognosis improved with the introduction of ACE inhibitors in the 1990s, SRC remains a major risk factor for mortality in SSc; endothelin receptor antagonists might further improve patient outcomes
Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2–15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3–5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients. The prognosis of SRC substantially improved in the 1980s with the introduction of angiotensin-converting-enzyme inhibitors for rapid blood pressure control, with additional antihypertensive agents as required. However, the survival of patients with SRC can still be improved. Current patient survival is 70–82% at 1 year, but decreases to 50–60% at 5 years despite dialysis support. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for transplantation. In this Review, we discuss progress made in the identification and proactive management of patients at risk of SRC and make recommendations aimed at optimizing management for those who progress to chronic kidney failure.
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Y.A.S. is funded by an Egyptian government scholarship programme of joint supervision in collaboration with D.E.F and the Division of Rheumatology, David Geffen School of Medicine, UCLA, Los Angeles, California, USA. We thank Gabriel Valdivia (University of California, Los Angeles, USA) for administrative assistance.
D.E.F. received research funding and consultation fees, and is on the advisory boards for Actelion, Gilead Sciences Inc., United Biosource Corporation (UCB), Pfizer, and Novartis. Y.A.S., T.G.W. and P.C. declare no competing interests.
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Woodworth, T., Suliman, Y., Li, W. et al. Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 12, 678–691 (2016). https://doi.org/10.1038/nrneph.2016.124
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