Abstract: TH-PO697
Triad of Troubles: Rare Confluence of Three Glomerular Diseases
Session Information
- Glomerular Diseases: Case Reports - 1
October 24, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Boldridge, Jessica, Brown University, Providence, Rhode Island, United States
- Shah, Ankur, Brown University, Providence, Rhode Island, United States
- Chang, Anthony, University of Chicago Division of the Biological Sciences, Chicago, Illinois, United States
Introduction
Glomerulonephritis (GN) is an important cause of ESKD contributing to 10-15% of all cases in the United States. IgA nephropathy is the most common cause of GN. Detection is important as delay in treatment can cause significant morbidity with progression to dialysis.
Case Description
A 61 yo female with a PMH of Grave's disease, Sjogren's, Raynauds, extranodal marginal zone lymphoma of the lung treated with rituximab presented initially with hematuria, proteinuria. She had a previous history of IgA vasculitis with renal and skin manifestations including bilateral purpura, hematuria and 0.3g/g proteinuria with preserved GFR. Renal biopsy had shown mild to moderate mesangial expansion, focal endocapillary hypercellularity with fine granular 2+ IgA staining on IF consistent with mesangioproliferative IgAN. She was started on steroids but due to worsening proteinuria and rising Cr she was switched to mycophenolate (MMF) in 2017. This was held in 2021 due to pneumonia. In 2023, she had worsening proteinuria with re-initiation of steroids and MMF. Reiniation of MMF caused anemia, gastrointestinal and infectious complications. A repeat kidney biopsy was performed to assess disease activity and prognosis desmonstrating a single fibrocellular crescent, diffuse mesangial hypercellularity and vacuolated capillary loops with a subepithelial spike formation. There was no significant glomerular staining for IgA. EM demonstrated sub-epithelial electron dense deposits. She was started on rituximab for concomitant membranous glomerulopathy and ANCA vasculitis and avacopan due to her prior steroid complications.
Discussion
We present a rare case of 3 coexisting biopsy proven glomerulopathies in one patient. This is a classic example of Hickam’s dictum, an aphorism that notes that the desire to find a single diagnosis for a patients ailments ignores the possibility of multiple co-existing disease states. In this case, the propensity for auto-immunity with her prior sjogrens, graves, and raynauds prompted consideration of multiple syndromes, which was found on biopsy. Treatment of multiple co-existing glomerulopathies is not well described, and our medical decision making was to attempt to apply occam’s razor to treatment, using a single treatment strategy that would treat all the co-existing glomerulopathies. This case demonstrates a wide range of medical decision making, highlighting its educational merit.