Introduction Collagen IV-related nephropathies, caused by mutations in the three genes encoding collagen type IV of the basal membrane, represent a spectrum of phenotypes from Alport syndrome (AS) to thin basement membrane nephropathy (TBMN) (1). Subtypes are X-linked AS (XLAS) which corresponds approximately 80% of AS, autosomal recessive AS (ARAS), counting for 15% of AS, and the autosomal dominant forms, represented by autosomal dominant AS (ADAS, 5% of AS) and TBMN, which is believed to be present in 1% of the population (1).