Rare Kidney Disease: Advances in Early Detection, Precision Therapies, and Clinical Practice Shifts With Swati Arora, MD

Despite the availability of biomarkers and advanced tools, early detection of rare kidney diseases remains challenging, and gaps in specialized training leave patients at risk for significant kidney damage.1

Rare kidney diseases affect approximately 60 to 80 individuals per 100,000 people, encompassing > 150 distinct conditions. They represent 5% to 10% of all chronic kidney disease (CKD) cases but disproportionately contribute to kidney failure, causing > 25% of cases requiring kidney replacement therapy.2

“Kidney disease is rare, which presents several challenges. During training, clinicians typically see only a small number of patients, often following them for just two years, resulting in limited longitudinal experience,” said Swati Arora, MD, FASN, an assistant professor at Drexel University, in an interview with HCPLive. “That’s why specialized education is so important. In recent years, more global nephritis fellowships and glomerulonephritis centers have emerged to support community nephrologists and other physicians in caring for patients with rare kidney diseases.”

The treatment landscape for rare kidney diseases has shifted toward precision medicine, targeting pathways such as galactose-deficient IgA and complement activation. While these therapies offer more targeted options than traditional broad immunosuppression, clinical understanding and experience remain limited due to gaps in specialized training.

Therapeutic options for rare kidney diseases now include targeted approaches such as B-cell and plasma cell therapies, complement inhibitors, endothelin receptor antagonists, and other emerging agents. Updates to clinical guidelines in 2021 and 2025 have supported earlier patient identification and more informed, evidence-based treatment decisions.

Clinical practice is also evolving. The threshold for performing kidney biopsies has been lowered. Whereas biopsies were once recommended only for proteinuria > 1 gram per day, recent data indicate that patients with proteinuria < 500 mg/day may still progress to end-stage kidney disease within a decade. This has prompted clinicians to intervene earlier in the disease course.

Looking ahead, Arora expresses growing optimism about noninvasive biomarkers to monitor disease activity, long-term data on targeted therapies, and innovative treatments designed to prevent progression to end-stage kidney disease or the need for transplant. The management of rare kidney diseases is advancing rapidly, with an emphasis on early detection, precision medicine, and improving long-term patient outcomes.

References

1. Rare Kidney Diseases Toolkit - International Society of Nephrology. International Society of Nephrology. Published January 29, 2026. Accessed February 24, 2026. https://www.theisn.org/initiatives/rare-kidney-diseases-toolkit/?utm_source=Facebook&utm_medium=Organic_social&utm_campaign=Toolkits#KidneyHealth

2. 6 New Kidney Disease Medications Approved in 2025. National Kidney Foundation. Published January 21, 2026. https://www.kidney.org/news-stories/6-new-kidney-disease-medications-approved-2025