Abstract: FR-PO0428
Complement-Mediated Thrombotic Microangiopathy (TMA) Triggered by Systemic Lupus Erythematosus (SLE): Favorable Response to Eculizumab
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
- Kaur, Rupinder, University of Southern California, Los Angeles, California, United States
- Taylor, Joseph, University of Southern California, Los Angeles, California, United States
- Shan, Hui Yi, University of Southern California, Los Angeles, California, United States
Introduction
Not all TMA in Autoimmune disease is atypical HUS or Comlement mediated TMA. In genetically susceptible individuals, complement activation may not be controlled and cause aHUS. Persistent TMA despite aggressive therapy targeting the autoimmune diseases should raise suspicion of aHUS. In addition to controlling the autoimmunity, terminal complement blockade should be considered.
Case Description
41 y F Patient with no reported past medical History admitted with fatigue, head ache and blurry vision. she was diagnosed with PRES (posterior reversible encephalopathy syndrome) and was also found to have pericardial effusion, bilateral pleural effusions, and ascites. Initial work up was negative. She was treated with antihypertensives and was discharged home. She later presented with AKI, Anemia and Thrombocytopenia . Lab work up showed schistocytes, low C3, C4 with significantly positive ANA however Serology was negative for SLE, APLS and negative ADAMTS13 . Renal Biopsy showed TMA however no evidence of Immune complex(IC) mediated Glomerular disease. She was treated with intravenous and oral steroids, Cellcept with partial improvement and finally with eculizumab with significant improvement in her symptoms and renal function. Mutation analysis resulted positive for pathogenic variant in CFI and patient was initiated on long term anti complement therapy.
Discussion
TMA in SLE has been reported in < 1% to 9% of affected patients. Non-immune complex(IC) mediated renal TMA has been reported in patients with SLE. Overlapping features of SLE and TMA make the diagnosis challenging. Presence of pathogenic complement gene mutation increase susceptibility to uncontrolled activation of complements leading to complement mediated TMA which showed favorable prognosis with Terminal complement blockade. Randomized controlled trials are required to investigate the utility of anticomplement therapies in TMA associated with autoimmune diseases.
TMA