Goodpasture's 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 haemorrhage. Simultaneous haemoptysis 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's disease, with the remainder mostly antineutrophil cytoplasmic antibody vasculitis.
Rapid diagnosis by early renal biopsy and serological 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’s disease, also known as anti-glomerular basement membrane antibody (anti-GBM) disease, is an important cause of pulmonary-renal syndrome (Goodpasture’s syndrome). Pulmonary-renal syndrome consists of glomerulonephritis and pulmonary haemorrhage. Goodpasture’s 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 sex
- 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
- anti-glomerular basement membrane (anti-GBM) antibody titre
- anti-neutrophil cytoplasmic antibodies (ANCA)
- serum complement (C3 and C4)
- antinuclear antibody
- hepatitis panel
- anti-streptolysin O titre
- clotting screen
reversible renal disease or any pulmonary involvement
irreversible renal disease
Richard G. Phelps, BChir
Senior Lecturer and Honorary Consultant in Nephrology
University of Edinburgh and Edinburgh Royal Infirmary
RGP has helped Genzyme develop educational materials for neurologists regarding the increased risk of Goodpasture's disease with alemtuzumab use. RGP has published an article on post-alemtuzumab nephritis in which employees of Genzyme were co-authors.
Dr Richard G. Phelps would like to gratefully acknowledge Professor Andre A. Kaplan and Dr Michael S. Gersch, previous contributors to this topic. AAK has received honoraria in 2013 to speak about plasma exchange from Gambro and Alexion (Gambro manufactures products for plasma exchange and Alexion manufactures a complement cascade inhibitor). MSG declares that he has no competing interests.
Dorin-Bogdan Borza, PhD
Assistant Professor of Medicine
Division of Nephrology
Department of Medicine
Vanderbilt University School of Medicine
DBB declares that she has no competing interests.
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