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Alport Early RASi Prophylactic — ESENeph MCQ

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HardGlomerulonephritisAlport Early RASi ProphylacticESENeph

A 30-year-old man presents with haematuria and proteinuria. He has hearing loss and his mother has CKD. Kidney biopsy shows thin and thick GBM segments with characteristic basket-weave splitting on EM. Genetic testing reveals a COL4A5 mutation. He is started on Ramipril. At what proteinuria level should Ramipril be started in Alport syndrome?

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Correct answer: B — Expert consensus suggests starting RASi at any level of proteinuria in X-linked Alport syndrome (COL4A5 mutations), even before significant proteinuria develops in males, to delay ESKD

The EARLY PRO-TECT Alport trial and expert consensus (Alport Syndrome Research Collaborative) recommend early initiation of RASi in X-linked Alport syndrome males, even with mild or absent proteinuria, to delay progression to ESKD. The rationale is that COL4A5 mutations cause progressive GBM damage, and haemodynamic protection with RASi started early can delay the inevitable progression by years to decades. This is one of the few situations where RASi is recommended prophylactically rather than reactively.

Reference: Gross et al 2012 – EARLY PRO-TECT; Kashtan 2021 – Alport Syndrome