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Fabry CKD Dual Management — ESENeph MCQ

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ModerateChronic Kidney DiseaseFabry CKD Dual ManagementESENeph

A 45-year-old woman with Fabry disease and proteinuric CKD G3a is on enzyme replacement therapy (Agalsidase beta). Her proteinuria is 1.2 g/day. What additional renoprotective measure should be prescribed?

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Correct answer: C — ACEi or ARB for proteinuria reduction – standard CKD renoprotective measures apply alongside disease-specific therapy

Fabry disease management requires both disease-specific therapy (ERT or chaperone therapy) AND standard CKD renoprotective measures. KDIGO and Fabry-specific guidelines recommend ACEi/ARB for proteinuria reduction. SGLT2i should also be considered based on CKD guidelines. The principle is that disease-specific treatment addresses the storage disorder while CKD therapies manage the downstream consequences (proteinuria, hypertension, CKD progression). These approaches are complementary, not alternative.

Reference: ERA-EDTA 2022 – Fabry Guideline; NICE HST4 2017