Anti-glomerular basement membrane (Anti-GBM) diseases, also known as anti-GBM antibody disease or Goodpasture's syndrome, refer to a group of autoimmune disorders that primarily affect the kidneys and lungs. These diseases are characterized by the presence of antibodies that target the glomerular basement membrane – a component of the kidney – causing destructive inflammation and tissue damage.
In Anti-GBM diseases, the immune system mistakenly produces antibodies against the glomerular basement membrane, leading to the formation of immune complexes. These complexes subsequently trigger an inflammatory response, primarily in the kidney and/or lung tissues. The antibody-mediated inflammation can result in rapid and severe damage to the glomeruli (clusters of tiny blood vessels in the kidney), leading to acute glomerulonephritis and kidney dysfunction. Additionally, the lung involvement in some cases can result in rapid and progressive lung hemorrhage, leading to respiratory distress.
Clinically, Anti-GBM diseases may present with symptoms such as hematuria (blood in urine), proteinuria (excess protein in the urine), cough, shortness of breath, fatigue, and generalized symptoms like fever and weight loss. Prompt diagnosis is crucial, typically involving blood and urine tests to detect the presence of anti-GBM antibodies and evaluate kidney and lung function. Biopsy of affected tissues may be performed to confirm the diagnosis.
Treatment for Anti-GBM diseases usually involves a combination of immunosuppressive medications, such as corticosteroids and cyclophosphamide, to reduce the immune response and prevent further damage. Plasmapheresis, a procedure to remove harmful antibodies from the blood, may also be initiated to rapidly decrease antibody levels. In severe cases, kidney transplantation or dialysis may be necessary for patients with advanced kidney failure
