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Fabry Disease RASi Renoprotection — ESENeph MCQ

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ModerateChronic Kidney DiseaseFabry Disease RASi RenoprotectionESENeph

A 39-year-old woman with known Fabry disease (alpha-galactosidase A deficiency) has proteinuria (uACR 15 mg/mmol) and eGFR 82 mL/min/1.73m2. Renal biopsy shows characteristic zebra bodies (myelin-like lamellated inclusions) in podocytes on electron microscopy. She is not on enzyme replacement therapy. Besides enzyme replacement, what additional renoprotective medication should be started?

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Correct answer: E — Ramipril

Fabry disease is an X-linked lysosomal storage disorder causing progressive CKD. KDIGO and Fabry disease guidelines recommend early RASi (ACEi or ARB) initiation for renoprotection alongside enzyme replacement therapy (agalsidase alfa or beta) or oral chaperone therapy (migalastat). Even with modest albuminuria (uACR 15 mg/mmol), RASi should be started to reduce proteinuria and slow CKD progression. The approach mirrors general CKD management principles. SGLT2i may offer additional benefit but evidence in Fabry-specific CKD is more limited compared to RASi.

Reference: Ortiz et al 2018 – European Fabry Working Group Recommendations; Warnock et al 2015 – Fabry Renal Outcomes