Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. 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. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for transplantation. Current patient survival is 70–82% at 1 year, but decreases to 50–60% at 5 years despite dialysis support. However, the survival of patients with SRC can still be improved. 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. 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. 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. 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 should be differentiated from ANCA-positive, rapidly progressive glomerulonephritis, as treatment regimens and patient management are differentĬontrol 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 mortalityĪlthough 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 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 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% Renal dysfunction associated with vasculopathy is a common pathology in systemic sclerosis (SSc), and usually exhibits a benign course
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