Anti-glomerular basement membrane disease

Anti-glomerular basement membrane (anti-GBM) disease, also known as Goodpasture disease or Goodpasture syndrome, is a rare autoimmune disorder in which the body produces autoantibodies directed against the alpha-3 chain of type IV collagen, a structural protein found in the basement membranes of the kidneys and lungs. This autoimmune attack leads to rapidly progressive glomerulonephritis (severe inflammation of the kidney's filtering units) and, in many cases, pulmonary hemorrhage (bleeding in the lungs). The disease primarily affects two organ systems: the kidneys and the lungs. When both organs are involved, the condition is classically referred to as Goodpasture syndrome. Patients typically present with rapidly worsening kidney function, which may manifest as blood in the urine (hematuria), protein in the urine (proteinuria), decreased urine output, and swelling. Pulmonary involvement causes cough, shortness of breath, and hemoptysis (coughing up blood), which can be life-threatening. Lung hemorrhage is more common in individuals who smoke or have had recent respiratory infections or exposure to inhaled toxins. The disease has a bimodal age distribution, with peaks in young adults (20–30 years) and older adults (60–70 years), and affects males more frequently than females. Diagnosis is confirmed by detecting circulating anti-GBM antibodies in the blood and characteristic linear immunoglobulin G (IgG) deposition along the glomerular basement membrane on kidney biopsy. Treatment requires urgent intervention and typically involves a combination of plasmapheresis (plasma exchange) to remove circulating antibodies, immunosuppressive therapy with corticosteroids and cyclophosphamide to suppress further antibody production, and supportive care including dialysis if kidney failure occurs. Early diagnosis and treatment are critical, as delays can lead to irreversible kidney damage requiring long-term dialysis or kidney transplantation. Relapses are uncommon but can occur, and long-term monitoring is recommended.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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