Goodpasture disease is defined by the presence of autoantibodies to the alpha-3 chain of type IV collagen. It presents with rapidly progressive renal dysfunction often combined with lung hemorrhage. Simultaneous hemoptysis and renal failure (the pulmonary renal syndrome) has many causes, but where the renal disease is a rapidly progressive glomerulonephritis, about 15% of cases will be Goodpasture disease, with the remainder mostly antineutrophil cytoplasmic antibody vasculitis.
Rapid diagnosis by early renal biopsy and serologic testing is essential, because appropriate treatment given before renal damage is advanced can result in an excellent prognosis.
Treatment is by removal of circulating antibodies using plasma exchange combined with agents to prevent their continued production.
Goodpasture disease, also known as antiglomerular basement membrane antibody (anti-GBM) disease, is an important cause of pulmonary-renal syndrome (Goodpasture syndrome). Pulmonary-renal syndrome consists of glomerulonephritis and pulmonary hemorrhage. Goodpasture disease is caused by an autoantibody to the alpha-3 chain of type IV collagen. This type of collagen is principally found in the basement membranes of alveoli and glomeruli.
History and exam
Key diagnostic factors
- reduced urine output
Other diagnostic factors
- male gender
- age 20 to 30 years or 60 to 70 years
- shortness of breath
- crackles on lung examination
- HLA-DRB1 or DR4
1st investigations to order
- renal function testing
- renal biopsy
- antiglomerular basement membrane (anti-GBM) antibody titer
- antineutrophil cytoplasmic antibodies (ANCA)
- serum complement (C3 and C4)
- antinuclear antibody
- hepatitis panel
- antistreptolysin O titer
- clotting screen
reversible renal disease or any pulmonary involvement
irreversible renal disease
- Systemic lupus erythematosus
- Granulomatosis with polyangiitis (Wegener)
- Microscopic polyangiitis
- Guidelines on the use of therapeutic apheresis in clinical practice
- KDIGO clinical practice guideline for glomerulonephritis
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