From Fibrosis to Failure: A Severe Case of Scleroderma Renal Crisis

Introduction: Systemic sclerosis (SSc) is an autoimmune disease causing vasculopathy and fibrosis. Scleroderma renal crisis (SRC) presents with hypertension (HTN), anemia, thrombocytopenia, and rapidly progressing acute kidney injury (AKI). Case Description: A 45-year-old Caucasian woman presented with worsening chest pain. History included heart failure, HTN, suspected connective tissue disease with recent anemia and thrombocytopenia. On exam, she had tight perioral skin, sclerodactyly, and elbow contractures. She was found to have hypertensive emergency (213/118). Labs revealed AKI with 2.55 mg/dL creatinine, BNP 2692, and proteinuria. ANA and RNA polymerase III (RP3) IgG were positive. Renal biopsy showed thrombotic microangiopathy consistent with SRC. She received ACE inhibitors, plasmapheresis for 5 days, and captopril 50 mg TID, with improved renal function. Discussion: SRC affects 5–15% of SSc patients, typically early in diffuse cutaneous forms. Risk factors include rapid skin thickening, anti-RP3 antibodies, and corticosteroid use. Pathogenesis involves endothelial injury, RAAS activation, and malignant hypertension. ACE inhibitors remain first-line therapy. Complement inhibition with eculizumab shows variable benefit; further studies are warranted.Light microscopy showed global glomerulosclerosis, ischemic hypoperfusion, onion-skinning of arteriolesElectron microscopy shows endothelial swelling, thin BM and no immune complex deposition.