There is no cure for XL Alport syndrome but treatment with renin-angiotensin-aldosterone (RAAS) blockade and SGLT2 inhibitors (in adults; safety and efficacy are not yet proven in children) delays kidney failure. Affected males with XL Alport syndrome should be treated from the time of diagnosis with RAAS blockade (typically ramipril, even in children) and SGLT2 inhibitors if necessary to control proteinuria.
Affected females with XL Alport syndrome should be treated from the onset of microalbuminuria. The aim is to minimise the urinary albumin level rather than to lower the blood pressure and the RAAS blockade can be increased as tolerated.
In addition people with Alport syndrome should stop smoking, not become overweight or develop diabetes, have their blood pressure controlled, and exercise regularly.
COL4A5