goodpasture syndrome
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:
- pulmonary hemorrhage
- glomerulonephritis
- circulating antiglomerular basement membrane antibodies
Epidemiology
It typically affects young adult men with an M:F of 3:1.
Clinical presentation
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.
Pathology
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.
Radiographic features
Plain radiograph
- 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
CT
- 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.
Differential diagnosis
Often radiograph and CT findings are indistinguishable from pulmonary edema. Other causes of pulmonary hemorrhage should also be considered.
See also
Siehe auch:
und weiter:
- verkalkte Lungenherde
- Vaskulitis
- pulmonale Vaskulitis
- Hämosiderose der Lunge
- adult chest radiograph common exam pathology
- Siderose
- acute unilateral airspace opacification
- acute bilateral airspace opacification
- Varizellenpneumonie
- diffuse Alveolarblutung
- IgG4-Autoimmunerkrankungen
- diffuse Lungenblutung
- Schmetterlingskonfiguration von Lungenverdichtungen
Assoziationen und Differentialdiagnosen zu Goodpasture-Syndrom:
