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Progressive nephropathy is one of the main features of <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content> and is marked by an insidious development, with an overall rate of progression of chronic kidney disease (CKD) very similar to diabetic nephropathy. Untreated patients usually develop end stage renal disease in their 50s. The decline in renal function in <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content> is adversely affected by male gender, advanced <jats:styled-content style=\"fixed-case\">CKD<\/jats:styled-content>, hypertension and, in particular, severe proteinuria. Enzyme replacement therapy (<jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>) has been shown to slow the progression of Fabry nephropathy. 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