Medicine (Baltimore). 2026 Feb 27;105(9):e47912. doi: 10.1097/MD.0000000000047912.
ABSTRACT
RATIONALE: Immunoglobulin A nephropathy (IgAN) and membranous nephropathy (MN) are common primary glomerular diseases. Although these conditions are well-characterized, their co-occurrence with Fabry-like zebra bodies is exceptionally rare. Fabry-like zebra bodies, characterized pathologically by numerous myeloid bodies within glomerular podocytes, are a hallmark feature of classical Fabry disease. However, similar ultrastructural findings can also arise from other genetic disorders or drug-induced phospholipidosis (e.g., associated with hydroxychloroquine or amiodarone).
PATIENT CONCERNS: The patient was a 51-year-old woman with clinical manifestations dominated by massive proteinuria and normal creatinine levels.
DIAGNOSES: The patient was diagnosed with IgAN and MN with Fabry-like zebra bodies.
INTERVENTIONS: Treatment comprised angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers.
OUTCOMES: After 1.5 years, she exhibited considerable decreases in creatinine from 61 μmol/L to 42.9 μmol/L and proteinuria from 3.8 g/24 h to 52.5 mg/24 h. Overall, the patient's clinical course was relatively smooth. Despite transient fluctuations in renal parameters, the patient achieved significant remission of proteinuria and edema following treatment.
LESSONS: This case highlights the complexity of diagnosing and managing the co-occurrence of IgAN, MN, and Fabry-like zebra bodies, emphasizing the need for further research on its pathogenesis, treatment, and prognosis.
PMID:41759999 | PMC:PMC12956247 | DOI:10.1097/MD.0000000000047912