Proteinuria severity in Alport spectrum subtypes

Alport syndrome (AS) is the most common inherited glomerular disease,1 caused by pathogenic (P) or likely pathogenic (LP) variants in three collagen IV genes: COL4A5, which leads to X-linked AS (XLAS), and COL4A3 and COL4A4, responsible for autosomal dominant AS (ADAS), and autosomal recessive AS (ARAS). Regarding the term ADAS, it is widely recognized that it does not accurately reflect the underlying disease.2 A recent publication based on international consensus has proposed the term Alport Risk-COL4A3 or COL4A4.