Abstract: TH-PO0536
Fulminant Crescentic IgAN with Renal-Limited Thrombotic Microangiopathy Progressing to Kidney Failure Despite Complement Inhibition
Session Information
- Glomerular Diseases: IgAN, IgA Vasculitis, and More
October 22, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Lazar, Ionela, Institutul Clinic Fundeni, Bucharest, Romania
- Borjoiu, Ioana, Institutul Clinic Fundeni, Bucharest, Romania
- Lujinschi, Stefan Nicolaie, Institutul Clinic Fundeni, Bucharest, Romania
- Ismail, Gener, Institutul Clinic Fundeni, Bucharest, Romania
Introduction
IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide and a major cause of kidney failure in young adults. In severe forms, IgAN may present with rapidly progressive glomerulonephritis (RPGN), crescentic lesions, and renal-limited thrombotic microangiopathy (TMA), all associated with poor prognosis. However, evidence regarding optimal therapeutic strategies and the role of complement inhibition in crescentic IgAN and renal-limited TMA remains limited.
Case Description
We report the case of a 36-year-old female with no significant past medical history who presented with macroscopic hematuria, nephrotic-nephritic syndrome (24h proteinuria 5.7 g/24h, serum albumin 2.3 g/dL), and RPGN (serum creatinine increasing from 1.2 to 2.33 mg/dL within 2 months). Immunologic workup was negative, with only mild C3 consumption. There was no evidence of systemic TMA or extrarenal features suggestive of systemic vasculitis. Kidney biopsy showed mesangial IgA deposition with crescentic glomerulonephritis (M1E1S1T1C2) and TMA lesions, including subendothelial fibrin deposition, focal endotheliosis, and arteriolar medial thickening. Electron microscopy (EM) did not identify electron-dense deposits. Due to the rapidly progressive course, persistent nephrotic-nephritic syndrome, recurrent gross hematuria, resistant hypertension, and severe hypervolemia, immunosuppressive therapy was progressively intensified with high-dose corticosteroids, cyclophosphamide, rituximab, plasma exchange, and complement inhibition with ravulizumab. Despite aggressive multimodal therapy, kidney replacement therapy was required after 8 months of treatment.
Discussion
This case highlights a fulminant IgAN phenotype with both RPGN and renal-limited TMA. The lack of systemic TMA, extrarenal vasculitic features, clear systemic complement activation, and EM-detectable deposits suggests a non-classical pattern driven by endothelial injury. Progression to kidney failure despite maximal immunosuppression, plasma exchange, and complement inhibition shows the lack of clear therapeutic options in this setting. Fulminant crescentic IgAN with renal-limited TMA may progress to kidney failure despite maximal therapy, highlighting the need for better markers to guide treatment.