Abstract: TH-PO0517
Occam's Razor or Hickam's Dictum: Systemic Autoimmune Disarray
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
- Doddi, Akshith, Brigham and Women's Hospital, Boston, Massachusetts, United States
- Shah, Sujal I., Brigham and Women's Hospital, Boston, Massachusetts, United States
- Chowdhury, Raad Bin Zakir, Brigham and Women's Hospital, Boston, Massachusetts, United States
Introduction
Minimal change disease (MCD) is a well-known pattern of glomerular injury and is known to have a wide gamut of potential etiologies. We present a case of MCD in the setting of systemic autoimmune disarray.
Case Description
A 62 year-old female with a history of autoimmune hypothyroidism, chronic neutropenia, and chronic sicca symptoms presented with proteinuria (urine protein/creatinine ratio 6.62 g/g; urine albumin/creatinine ratio above limit of detection) and hypoalbuminemia (albumin 1.6 g/dL) in the setting of developing fatigue, an approximately 10 pound weight gain, lower extremity edema, and frothy urine of multiple weeks duration. Her clinical course in the prior four years has been significant for RAS-associated autoimmune lymphoproliferative disorder (RALD), confirmed by bone marrow biopsy and pathogenic cytogenetics notable for NRAS G12D mutation (variant allele fraction 43.7%) in the setting of Sjogren’s disease with associated pericarditis and neuromyelitis optica spectrum disorder (NMOSD). She underwent long-term surveillance with her hematology team and was maintained on complement inhibition for NMOSD. She was referred to nephrology for the nephrotic syndrome; workup was notable for hematuria, a faint IgGκ paraproteinemia, and positive anti-dsDNA, rheumatoid factor, ANA, SSA, and SSB antibodies. In this setting, the patient underwent a kidney biopsy. Her kidney biopsy was notable for evidence of anti-nephrin-negative diffuse podocytopathy, strong polyclonal anti-nuclear (ANA) tissue activity, and tubuloreticular inclusions in the glomerular endothelial cells. In this clinical setting, the presence of the strong IgG tissue ANA further raised the concern for an autoimmune process driving the diffuse podocytopathy. For this reason, therapy with a steroid taper and two infusions of obinituzumab was pursued. She has had marked improvements in her proteinuria (urine albumin/creatinine ratio became undetectable by one month after initiating therapy), serum albumin (normalization by three months after initiating therapy), and kidney function (returned to baseline).
Discussion
This case illustrates MCD in the setting of RALD and Sjogren’s disease with systemic manifestations. The attribution of etiology in this setting highlights the tension between the paradigms of Occam’s Razor and Hickam’s Dictum. Most notable in this case is the response of this patient’s glomerular injury to cytotoxic anti-CD20 therapy.