Abstract: FR-PO0435
A Case of Eculizumab Use in Severe Scleroderma Renal Crisis Thrombotic Microangiopathy
Session Information
- AKI: Case Reports - TMA, Vasculitis, Immune-Mediated Injury, and Systemic Disease
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Ramos, Kristina A., Lenox Hill Hospital, New York, New York, United States
- Patel, Anuj A., Lenox Hill Hospital, New York, New York, United States
- Rosenstock, Jordan L., Lenox Hill Hospital, New York, New York, United States
Introduction
Scleroderma renal crisis (SRC) is a severe complication of systemic sclerosis characterized by acute kidney injury (AKI), severe hypertension, and thrombotic microangiopathy (TMA). Emerging evidence suggests complement activation may contribute to refractory SRC-associated TMA, raising interest in complement inhibition as adjunctive therapy.
Case Description
A 79-year-old man with systemic sclerosis, hypertension, and positive RNA polymerase III antibodies developed progressive cutaneous thickening and extremity edema without Raynaud phenomenon. Prednisone was initiated for worsening skin disease, followed by methotrexate.
Two months later, he presented with dyspnea, edema, severe hypertension, AKI, nephrotic-range proteinuria, hematuria, anemia, and thrombocytopenia concerning for SRC-associated TMA. He had previously normal kidney function with baseline creatinine 0.64 mg/dL. Admission creatinine was 2.58 mg/dL and subsequently rose to 4.43 mg/dL despite improved blood pressure control with ACE inhibitor therapy. Laboratory evaluation demonstrated persistently elevated LDH, undetectable haptoglobin, and thrombocytopenia. Kidney biopsy showed active TMA consistent with SRC-associated endothelial injury.
Given ongoing renal deterioration, eculizumab was initiated 6 days after admission. Platelet count normalized within days and LDH progressively improved. Renal function stabilized and gradually recovered over several months, with creatinine improving to 1.45–1.6 mg/dL. Eculizumab was discontinued after 3 months. Three months after the final dose, creatinine remained stable at 1.4 mg/dL without recurrent TMA or dialysis requirement.
Discussion
This case illustrates severe steroid-triggered SRC complicated by biopsy-proven TMA. Although ACE inhibitors remain standard therapy, refractory SRC may involve complement-mediated endothelial injury. In this patient, complement blockade with eculizumab was associated with hematologic improvement and subsequent renal recovery, though delayed benefit from ACE inhibition cannot be excluded. This case supports a potential role for complement inhibition in selected patients with refractory SRC-associated TMA.