Fact-sheet: Goodpasture syndrome
Updated on 04/23/2019 at 9:50 AM
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Definition
-Rare autoimmune disease. -Preexisting autoantibodies directed against the glomerular basement membrane (anti-GBM).-Predisposing factor: injury (sensitization to antigens) to pulmonary or renal capillaries from organic solvents/hydrocarbons/viruses.. (occupational disease). Diagnosis based on clinical findings/imaging/antibody assay. - and antibodies directed against the NC1 domain of the alpha 3 chain of type IV collagen
Clinical features
Combination of:
- pulmonary involvement: hemoptysis / dry cough / dyspnea.
- renal involvement: rapidly progressive glomerulonephritis, hematuria, and renal failure.
Hence also referred to as "pulmonary-renal syndrome".
Laboratory findings
Renal failure.
Possible anemia.
Anti-GBM antibody testing (specific).
Radiography
Diffuse alveolar hemorrhage: poorly defined bilateral opacities.
Chest radiograph normal in 1/3 of cases.
Ultrasound
no
CT
Findings are identical to other causes of diffuse alveolar hemorrhage:
- ground-glass opacities.
- parenchymal consolidations with air bronchogram, sometimes fleeting and migratory.
- perihilar and dependent predominance.
- non-symmetric bilateral involvement.
- signs of chronic involvement: thickening of the interstitium, centrilobular micronodules, and interlobular septal thickening.
Management
Recurrent "idiopathic" diffuse alveolar hemorrhage with renal involvement should prompt renal biopsy.
Definitive diagnosis by renal biopsy.
Differential diagnosis
All other causes of diffuse alveolar hemorrhage and ground-glass opacity.
Clinical context is important++