Despite the severity of disease, the high rate of ESRD, and the increasing knowledge about genetics and diagnosis, there is currently no proven treatment to AS. So far, the therapeutic approach is limited to angiotensin-II (AII) blockade, blood pressure control and diet counseling. Angiotensin-converting enzyme inhibitors (ACEi) or angiotensin receptor blockers (ARB) are usually started once proteinuria appears but there is no clear evidence that such treatment alters the natural history of the disease in humans.