Angiotensin-converting enzyme inhibitors in patients with Alport syndrome: can all patients benefit?

Angiotensin-converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARBs) are prescribed to slow the progression of kidney disease in patients with Alport syndrome. In a recent publication by Yamamura et al. the authors showed an association of ACEi or ARB treatment with delay in ESKD, even for those patients with severe, truncating mutations. Despite these encouraging findings, there remain a number of clinical questions about the use of ACEi and ARBs in Alport syndrome.