Goodpasturesyndrom

Goodpasture syndrome, also known as anti-glomerular basement membrane disease, is an autoimmune disorder in which autoantibodies target the basement membranes of the kidneys and lungs. The condition classically presents with a combination of rapidly progressive glomerulonephritis and pulmonary hemorrhage, but the spectrum can vary from isolated kidney involvement to predominant lung disease.

Pathophysiology involves autoantibodies directed against the type IV collagen in the glomerular and alveolar basement membranes. This leads to inflammation and tissue injury in these organs, driven by a type II hypersensitivity mechanism. Clinically, patients may experience hemoptysis, dyspnea, and cough due to alveolar hemorrhage, as well as signs of nephritic syndrome such as hematuria, proteinuria, hypertension, and rapidly declining kidney function.

Diagnosis rests on clinical presentation plus serology and tissue findings. Anti-glomerular basement membrane antibodies are detected in the blood by immunoassays (often ELISA). Kidney biopsy frequently shows crescentic glomerulonephritis with linear deposits of IgG along the glomerular basement membrane on immunofluorescence. In some cases, lung tissue or bronchoalveolar lavage may show alveolar hemorrhage associated with similar basement membrane involvement.

Treatment aims to remove circulating antibodies and suppress ongoing immune activity. The standard approach includes plasma exchange to deplete anti-GBM antibodies, combined with high-dose corticosteroids and cyclophosphamide. Supportive care may include management of renal failure with dialysis. Early recognition and treatment improve renal outcome and survival; untreated disease carries a high risk of mortality.

Epidemiology: the disease is rare, with a higher prevalence in males and typically presents in young adults, though cases can occur at various ages.