Treating a rare kidney disease: a physician’s perspective

Some kidney diseases are more common than others. What are some of the challenges of treating a rare kidney disease, such as IgA nephropathy or C3 glomerulopathy?

The primary challenge of treating rare kidney diseases is that they do not present with the same symptoms and course in every person. The course of treatment is often determined by the way the disease presents. Is it quickly progressing, is it very symptomatic with swelling, fatigue, shortness of breath, decreased appetite? Or is it slowly progressing and asymptomatic. The treatments involve immunosuppression that can have serious side effects. Evaluation of the risks and benefits, and making sure the person being treated understands, is a common challenge with the treatment.

In addition to a nephrologist, what other providers or specialists make up a healthcare team for a rare kidney disease?

There are different co-morbidities that people can develop with these rare diseases, like hypertension. A primary care provider is a key provider in addition to the nephrologist. And a cardiologist is another important specialist if the person has any other cardiac comorbidities. The primary cause of death in people with kidney disease is cardiovascular disease.

What can a person with a rare kidney disease do to keep their kidneys as healthy as possible? Do diet and nutrition play a role?

There often is nothing that can be done to reverse the cause of these rare diseases. What a person can do is limit their chances of developing additional comorbidities like high blood pressure and diabetes—ensuring they maintain a healthy weight and avoiding medications like NSAIDS and certain antibiotics that could cause additional injury to the kidneys.

What is the treatment process like for a rare kidney disease? What can a person do to get the most out of treatment?

There is no straight forward process to treating rare diseases. The first step is usually to run more tests to see if there are any other processes evolving in the person that instigated the glomerulopathy (for example, cancer). The second step is treatment, which can range from supportive measures and monitoring to chemotherapy-level immunosuppression. It requires constant monitoring—a person’s labs as well as signs and symptoms of progression—to determine the appropriate treatments at the right time.

Michael A. Fischman, MD is a nephrologist at Southland Renal Medical Group in Southern California.