Kidney Week

Abstract: TH-PO1116

Drug-Induced Phospholipidosis: When a Common Medication Leads to an Uncommon Pathology

Session Information

• Pathology and Lab Medicine
  October 22, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pathology and Lab Medicine

• 1700 Pathology and Lab Medicine

Authors

• Al Suradi, Haya H., The Johns Hopkins Hospital, Baltimore, Maryland, United States

Haya H. Al Suradi, MD

• Alahmadi, Ziad, The Johns Hopkins Hospital, Baltimore, Maryland, United States

Ziad Alahmadi, MBBS

• Wilkins, Reid C., The Johns Hopkins Hospital, Baltimore, Maryland, United States

Reid C. Wilkins, MD

• Abifaraj, Farah, The Johns Hopkins Hospital, Baltimore, Maryland, United States

Farah Abifaraj, MD

• Raman, Gaurav, The Johns Hopkins Hospital, Baltimore, Maryland, United States

Gaurav Raman, MD

Introduction

Drug-induced phospholipidosis (DIPL) is an underrecognized condition caused by cationic amphiphilic drugs (CADs) that accumulate within lysosomes, forming characteristic lamellar inclusions (myeloid bodies) on electron microscopy. These findings can mimic lysosomal storage disorders, particularly Fabry disease, leading to diagnostic uncertainty. Implicated medications include, but not limited to- amiodarone, gentamicin, chloroquine, and selective serotonin reuptake inhibitors (SSRIs). We present a case of incidentally identified sertraline-induced phospholipidosis.

Case Description

A 75-year-old man with biopsy-proven immune complex glomerulonephritis was admitted with septic shock due to a necrotizing soft tissue infection and developed acute kidney injury (creatinine 2.4 mg/dL from a baseline of 1.8–2.0). Proteinuria increased from 0.7 g pre-hospitalization to 2.1 g, with a bland urinalysis. Repeat kidney biopsy showed stable, less extensive mesangiopathic changes and acute tubular injury consistent with ischemia. Electron microscopy revealed lysosomal inclusions with myelin bodies, absent on prior biopsy. Although suggestive of Fabry disease, the patient lacked clinical features or family history. Given the temporal association with SSRI initiation, sertraline was identified as the likely cause of DIPL.

Discussion

CADs inhibit lysosomal phospholipases, leading to phospholipid accumulation that mimics Fabry disease. Unlike Fabry disease, DIPL occurs without α-galactosidase A deficiency and typically demonstrates a polyclonal distribution. DIPL may worsen proteinuria and kidney disease progression, potentially in a duration-dependent manner, and may be reversible with drug discontinuation. This case highlights the importance of recognizing DIPL as a Fabry mimicker and underscores the need for careful medication review to avoid unnecessary enzymatic and genetic testing.