Context:

IgA nephropathy (IgAN) is a lifelong autoimmune kidney disease where IgA antibodies accumulate in the glomeruli, causing inflammation and leakage of blood and protein into urine. The condition varies widely among individuals, with some living symptom-free for years and others progressing to kidney failure. There is no cure, but treatments aim to slow progression, control blood pressure, and manage symptoms, while lifestyle and dietary changes support kidney protection. Diagnosis relies on exams, lab tests, and a kidney biopsy, and management combines medications, potential trials, and, in severe cases, transplant options. Ongoing research explores new therapies and individualized care pathways to delay or prevent complications.

Dive Deeper:

• IgA nephropathy is categorized into primary, secondary, and IgA-associated nephropathy, based on where IgA deposits occur and whether an underlying condition drives the process. Primary IgAN involves excessive galactose-deficient IgA1 (Gd-IgA1) with antibodies forming immune complexes in the kidneys. Secondary IgAN arises when another disease, such as liver disease or an autoimmune condition, impairs IgA clearance, presenting with both IgAN and the underlying condition.

• Diagnosis begins with physical examination and symptom review (blood in urine, swelling, high blood pressure). It relies on urinalysis and blood tests to assess kidney function, plus a kidney biopsy with immunofluorescence to confirm IgA deposits and determine disease type and extent.

• Treatment centers on slowing progression and alleviating symptoms. First-line medicines include ACE inhibitors or ARBs to reduce proteinuria and protect kidney function, with SGLT2 inhibitors and endothelin receptor antagonists as newer options. Corticosteroids and targeted therapies (e.g., complement inhibitors or APRIL-blocking antibodies) are considered based on disease activity and risk.

• Surgical approaches are not standard but may include tonsillectomy or kidney transplant in select cases; tonsil removal has shown variable impact on IgAN progression, while transplantation remains an option though recurrence in a transplanted kidney is possible.

• Prognosis varies; about half of patients may develop kidney failure within 10 years of diagnosis, though many respond to treatment and maintain kidney function longer. Chronic kidney disease, nephrotic syndrome, high blood pressure, and cardiovascular risk are notable complications to monitor, with ongoing research and clinical trials exploring new therapies and personalized care.