Abstract: FR-PO0702
Pseudo-Fabry Nephropathy: When Myeloid Bodies Mislead
Session Information
- Glomerular Diseases: Membranous Nephropathy, FSGS, and Podocytopathies
October 23, 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
- Mkrtchyan, Norayr, University of California Davis, Sacramento, California, United States
- Ananthakrishnan, Shubha, University of California Davis, Sacramento, California, United States
Introduction
Fabry’s Disease is a lysosomal storage disease that results in accumulation of glycosphingolipids due to deficient lysosomal alpha-galactosidase A with various manifestations. Pseudo-Fabry disease describes similar renal phospholipidosis on biopsy, but in the absence of alpha-galactosidase A deficiency. We present a case of sertraline-associated pseudo-Fabry nephropathy discovered during evaluation of proteinuria.
Case Description
A 42-year-old woman was referred for evaluation of proteinuria detected during workup for fatigue. Relevant history includes anxiety, depression, PTSD, IBS, and hypothyroidism. Medication list includes sertraline, bupropion, levothyroxine, and acyclovir. Urinalysis showed 1+ blood and 2+ protein with 1.2 g of proteinuria on a 24-hour urine collection. Serologic work-up was negative.
A kidney biopsy showed largely unremarkable findings on light microscopy and immunofluorescence, however electron microscopy revealed alternating thin and thick glomerular basement membranes with myeloid bodies within the podocyte cytoplasm. This patient’s presentation did not appear to be consistent with hereditary disease, given lack of systemic symptoms, negative family history, and negative genetic testing for Fabry and COL4A. Given chronic sertraline exposure and the known association between cationic amphiphilic drugs and lysosomal phospholipid accumulation, findings were favored to represent sertraline-induced pseudo-Fabry disease. Anti proteinuric therapy was started while continuing the sertraline (due to patient preference) with improvement in proteinuria.
Discussion
Drug-induced renal phospholipidosis is an important mimic of Fabry disease. While hydroxychloroquine is the most commonly associated medication, among SSRIs, sertraline due to its strong cationic amphipilic drug properties, can induce a similar pathology. Recognition of pseudo-Fabry disease is essential to avoid misdiagnosis and unnecessary treatment. This case represents the necessity to correlate clinical findings, medication reconciliation, and analysis of genetic history to optimize treatment planning.