IgA Nephropathy
Urinary System Confirmed Autoimmune

IgA Nephropathy

Also known as: Berger's Disease, Immunoglobulin A nephropathy, IgAN

Primary organ/tissue: Kidneys

Overview

IgA nephropathy is a chronic autoimmune kidney disease where a protein called immunoglobulin A (IgA) builds up in the kidneys' filtering units (glomeruli). This buildup causes inflammation and damage, slowly impairing the kidneys' ability to filter waste and excess fluid from the body.

Symptoms

Blood in urine (hematuria) - either visible or microscopic; Foamy urine - from excess protein; Swelling - especially in the feet, hands, or face; High blood pressure; Fatigue; Lower back or flank pain. Often triggered or worsened by respiratory or gastrointestinal infections. Early stages often have no symptoms.

Causes

Caused by a faulty immune response involving abnormal IgA1, a type of antibody missing certain sugars (galactose-deficient IgA1), making it look foreign to the immune system. The body attacks it, forming immune complexes that get stuck in the kidneys and cause inflammation. Key contributors include genetics, immune system abnormalities, infections (particularly respiratory or GI tract infections), and environmental factors.

Diagnosis

Urinalysis - shows blood and protein in urine; Blood tests - check kidney function (creatinine, estimated GFR); Kidney biopsy - confirms IgA deposits in glomeruli; Imaging - may rule out other kidney problems. Diagnosis often happens after an unrelated urine test.

Treatment

No cure, but treatments aim to slow disease progression: Blood pressure medications (ACE inhibitors or ARBs); Corticosteroids or immunosuppressants (for high-risk patients); Fish oil supplements (may reduce inflammation); Low-salt, kidney-friendly diet; SGLT2 inhibitors (newer option in some patients); Kidney transplant for end-stage kidney disease.

Prognosis

IgA nephropathy progresses slowly in most people. About 1 in 4-5 will develop kidney failure over 20 years. Others live with stable or mild disease for decades. Controlling blood pressure and protein levels in urine are key to improving long-term outcomes.

Prevalence

Affects approximately 2 per 100,000 people per year. More common in men and young adults (teens to 30s). Higher rates in East Asian and Caucasian populations.

Autoantibodies

AutoantibodyNotes
Galactose-deficient IgA1 Not a classic autoantibody; involves autoantibodies against galactose-deficient IgA1, forming damaging immune complexes

Citations

  1. Wyatt RJ, Julian BA. IgA nephropathy. N Engl J Med. 2013;368(25):2402-14.
  2. Kiryluk K, Novak J. The genetics and immunobiology of IgA nephropathy. J Clin Invest. 2014;124(6):2325-32.
  3. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;100(4S):S1-S276.