Goodpasture syndrome, also referred as antiglomerular basement membrane (anti-GBM) antibody disease, is an autoimmune disease characterized by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. It is a type of pulmonary-renal syndrome.
Goodpasture syndrome is defined by:
It typically affects young adult men with an M:F of 3:1.
Patients may present with cough, dyspnea, hemoptysis and hypoxia. Pulmonary manifestations are usually the cause of presentation although most also have evidence of renal disease.
Type II hypersensitivity reaction with antibodies directed primarily against renal glomerular basement membrane collagen type IV and cross-reacts with alveolar basement membrane to produce the renal injury, usually rapidly progressive glomerulonephritis and necrotizing hemorrhagic interstitial pneumonitis characteristic of this disorder.
The diagnosis is made by immunofluorescent studies of renal or lung tissue, which show a smooth wavy line of fluorescent staining along the basement membrane.
- non-specific changes on chest radiographs
- bilateral, coalescent airspace opacities, which in several days resolve to give reticular opacities in the same distribution
- complete radiographic resolution is usually seen within 2-3 weeks
- ground glass and airspace opacities that progress to reticular "crazy paving" pattern over a few weeks
- hilar lymphadenopathy may be seen
- no interlobular septal thickening in the acute phase
Treatment and prognosis
The overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival.
- 1. Brant WE, Helms CA. Fundamentals of diagnostic radiology. Lippincott Williams & Wilkins. (2007) ISBN:0781765188. Read it at Google Books - Find it at Amazon
- 2. Primack SL, Miller RR, Müller NL. Diffuse pulmonary hemorrhage: clinical, pathologic, and imaging features. AJR Am J Roentgenol. 1995;164 (2): 295-300. AJR Am J Roentgenol (abstract) - Pubmed citation
- 3. Hansell DM. Small-vessel diseases of the lung: CT-pathologic correlates. Radiology. 2002;225 (3): 639-53. doi:10.1148/radiol.2253011490 - Pubmed citation
- 4. Adam A, Dixon AK, Gillard JH et-al. Grainger & Allison's Diagnostic Radiology: Expert Consult: Online and Print. Churchill Livingstone. ISBN:B00K8G3JYK. Read it at Google Books - Find it at Amazon
- 5. Mayberry JP, Primack SL, Müller NL. Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. Radiographics. 2000;20 (6): 1623-35. doi:10.1148/radiographics.20.6.g00nv031623 - Pubmed citation