From the Guidelines
Goodpasture syndrome is a rare autoimmune disorder characterized by the presence of anti-GBM antibodies, which attack the basement membranes of the lungs and kidneys, leading to a pulmonary-renal syndrome. This condition is caused by antibodies directed against the α3 chain of type IV collagen, present in the glomerular and alveolar basement membranes 1. The syndrome can present as a classical pulmonary-renal syndrome, known as Goodpasture syndrome, or as a renal-limited disease, referred to as anti-GBM nephritis.
Key Characteristics
- The immune system mistakenly attacks the basement membranes of both the lungs and kidneys
- Antibodies are produced against type IV collagen, found in these basement membranes
- Inflammation and damage occur in both organs, causing symptoms such as:
- Coughing up blood (pulmonary hemorrhage)
- Difficulty breathing
- Fatigue
- Kidney problems, including:
- Blood in urine
- Protein in urine
- Potentially kidney failure
Treatment and Prognosis
Treatment focuses on removing the harmful antibodies through plasma exchange (plasmapheresis) and suppressing the immune system with medications. Prompt recognition and early treatment are crucial to prevent kidney failure, which is inevitable if left untreated, with a mortality rate of up to 96% 1. With proper management, the prognosis has improved significantly in recent years, though some patients may still develop chronic kidney disease requiring dialysis or transplantation.
From the Research
Definition and Characteristics of Goodpasture Syndrome
- Goodpasture syndrome is a rare autoimmune disorder characterized by the presence of anti-glomerular basement membrane (anti-GBM) antibodies, leading to rapidly progressive glomerulonephritis and/or pulmonary hemorrhage 2, 3, 4, 5.
- The syndrome is caused by autoantibodies that bind to the noncollagenous domain of the alpha 3 chain of type IV collagen in the glomerular and alveolar basement membranes 2, 4.
- The autoantibodies activate the complement cascade, resulting in tissue injury through a type II hypersensitivity reaction 2.
Clinical Presentation and Diagnosis
- Patients with Goodpasture syndrome typically present with renal failure, hematuria, proteinuria, and hemoptysis 2, 5.
- The diagnosis is often made based on the presence of anti-GBM antibodies and kidney biopsy showing crescentic glomerulonephritis 2, 6.
- The syndrome can be life-threatening and requires prompt diagnosis and treatment to prevent organ damage and improve outcomes 3, 5.
Treatment and Prognosis
- Treatment typically involves a combination of plasmapheresis, corticosteroids, and immunosuppressive drugs such as cyclophosphamide 2, 3, 6, 5.
- Early and aggressive treatment is crucial to improve outcomes and prevent long-term organ damage 2, 3, 5.
- Prognostic factors include the degree of renal and lung damage at the time of presentation, as well as the patient's age and response to treatment 2, 6.
- New therapeutic options, such as Eculizumab and ECMO, may be effective in severe cases of Goodpasture syndrome 3.