Scleroderma Renal Crisis (SRC)



Clinical Presentation of Scleroderma Renal Crisis:

1) Acute kidney injury

2) Abrupt onset of hypertension

3) Abnormal urinalysis 

3) microangiopathic hemolytic anemia (MAHA)



Pathophysiology:

 • Precise pathogenesis is unknown but may involve vasculopathy, endothelial cell activation, ↓ perfusion

 • Biopsy → endothelial cell injury, arteriolar thickening, fibrinoid necrosis, fibrotic "onion-skinning" of arteries



Management Pearls:

 • Hospitalization for BP control & Monitoring

 • Renal Biopsy to confirm diagnosis and rule out other pathology (e.g. pauci-immune GN)

 • Initiate ACEi (short-acting, e.g. captopril, to allow titration q6 hours) for initial therapy.

 • If uncontrolled with ACEi, add other BP meds

 • Refractory cases → consider PLEX (overlap TTP or aHUS), eculizumab, vasodilators (ERAs)



By Mithu Maheswaranathan, MD @MithuRheum



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