In the two most recent DDD cohorts, reduced GFR at diagnosis was associated significantly on multivariate analysis with progression to ESKD.4, 21 In CFHR5 nephropathy, in which progressive disease is characterized by hypertension, proteinuria, and chronic renal failure, ESKD has been reported in 18 of 91 carriers (or 20%) of the heterozygous CFHR5 gene mutation. The gene discussed is CFHR5; the disease is Nephropathy.