Richard Lafayette, MD, FACP, Director of the Stanford Glomerular Disease Center, Editor-in-Chief of ASN Kidney News, and Rheumatologist at Stanford Health Care, describes the current standard of care for immunoglobulin A (IgA) nephropathy. 

IgA nephropathy, also known as Berger’s disease, is a rare kidney disease characterized by build up of IgA deposits in the kidneys.  The buildup of IgA deposits inflames and damages the glomeruli, causing hematuria and proteinuria. This damage may lead to scarring of the nephrons which progresses slowly over many years. Eventually, IgA nephropathy can lead to end-stage kidney disease. 

As Dr. Lafayette notes, there is currently no approved treatment for IgA nephropathy. However, there is a standardized approach outlined by the Kidney Disease Improving Global Outcomes (KDIGO) Guidelines. The first step in this approach is to get as early a diagnosis as possible. An official IgA nephropathy diagnosis can only be achieved through a kidney biopsy. The next step is to do a risk assessment on the patient. As Dr. Lafayette explains, signs a patient is at high risk include high levels of creatinine, substantial amounts of proteinuria (> 1000 mg per day), and high blood pressure, as well certain characteristics of the kidney biopsy such as significant inflammation or scarring. If a patient is considered high risk for progressing into kidney disease, they will put on a treatment regimen to avoid further kidney damage. This will likely include a diet of low-sodium foods and medication to control blood pressure by blocking the renin-angiotensin system. The use of corticosteroids has had varying success rates in controlling kidney inflammation in IgA nephropathy patients. According to Dr. Lafayette, some trials have shown corticosteroids can successfully reduce proteinuria levels and slow the loss of kidney function. However, other studies have shown no benefits of corticosteroids in these patients. Additionally, all studies have shown substantial risks of the continued use of corticosteroids which physicians should be aware of when treating IgA nephropathy patients.

The use of risky corticosteroids may be unnecessary soon as the FDA is scheduled to make a decision about the approval of Nefecon, a potential treatment for IgA nephropathy, by September 15, 2021. 

To learn more about IgA nephropathy and other rare kidney diseases, visit checkrare.com/diseases/kidney