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 #SRC #Scleroderma #Renal #Crisis #rheumatology #nephrology #diagnosis #management
