Plasmapheresis for Treatment of Renal Scleroderma Crisis

Introduction: Scleroderma renal crisis (SRC) is a rare and life-threatening complication of systemic sclerosis, occurring in approximately 4–6% of cases. It is characterized by abrupt-onset severe hypertension and acute kidney injury (AKI), often accompanied by microangiopathic hemolytic anemia. Although the pathophysiology remains complex, vasculopathy, endothelial dysfunction, and RAAS activation play central roles. Early treatment with ACE inhibitors has significantly improved outcomes. Plasmapharesis in addition to ACE inhibition has been associated with improved outcomes in comparison to ACE inhibitors only. Case Description: A 45-year-old Caucasian woman with HTN, HFpEF, and undifferentiated connective tissue disease presented with biopsy-proven thrombotic microangiopathy consistent with SRC. BP was 213/118 mmHg. She reported weakness and stiffness; exam showed thickened skin of fingers and toes. Baseline creatinine was 0.46 mg/dL; admission creatinine was 3.38 mg/dL. Urine protein was 1,968 mg/day. Serologies were negative. Anemia and thrombocytopenia were present. She was treated with captopril 50 mg TID, amlodipine 10 mg daily, and plasmapheresis. Creatinine improved with 5 sessions, and BP normalized. She was discharged on captopril 25 mg TID with close follow-up. Discussion: Thrombotic microangiopathy (TMA) includes a range of serious conditions, and identifying the underlying cause is essential for treatment. In this patient, malignant hypertension, acute kidney injury, cytopenias, and connective tissue disease strongly suggested SRC. Other causes such as TTP and HUS were considered but were less likely given normal ADAMTS-13 and negative autoimmune serologies. Renal biopsy showing TMA in the setting of systemic sclerosis supported the diagnosis. SRC management centers on blood pressure control with ACE inhibitors, particularly captopril. However, outcomes remain poor, with 5 year mortalility still at an unacceptable rate. In select patients, plasmapheresis has been explored as adjunctive therapy to remove circulating factors driving endothelial injury. Though data are limited, case series suggest combining plasmapheresis with ACE inhibition may improve renal recovery and outcomes. In this case, the dual approach led to rapid improvement and preserved renal function, supporting its potential role in SRC.