Medical Care: No definite treatment exists for AS. Data from animal studies suggest benefits from angiotensin-converting enzyme (ACE) inhibitors in the reduction of proteinuria and progression of renal disease; thus, the use of ACE inhibitors is reasonable in patients with AS who have proteinuria with or without hypertension. Some reports suggest that cyclosporine may reduce proteinuria and stabilize renal functions in patients with AS; however, the studies were small and uncontrolled. Larger and controlled studies are needed to define the role of cyclosporine in AS.
Gene therapy for Alport syndrome is being studied. Animal studies are underway to evaluate the delivery of human alpha-5 (IV) chain of GBM in a canine model of X-linked Alport syndrome.
Surgical Care: Renal transplantation is usually offered to patients with AS who develop ESRD. The allograft survival rate in these patients is similar to patients with other renal diseases. Recurrent disease does not occur in the transplant; however, approximately 3-5% of patients with AS who undergo transplant develop anti-GBM nephritis. In view of excellent graft survival rates and a very low incidence of anti-GBM disease, renal transplantation is not contraindicated in patients with AS.
Diet: Patients require a renal failure diet once ESRD ensues.