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Renal manifestations of systemic sclerosis

  • Scleroderma renal crisis (SRC) = abrupt onset of HTN (relative to patient’s baseline), MAHA. Urine sediment typically bland. Renal biopsy not required but would show “onion-skin” hypertrophy of arteries & arterioles.
  • Affects 5-10%. ACEI effective (see below) but 40% still progress to ESRD and 5-year mortality is → 40% (QJM 2007;100:485).
  • Risks: diffuse cutaneous systemic sclerosis (dcSSc), early disease (⅔ of cases in 1st yr), >15 mg/d prednisone, RNA Pol III Ab.