ANTI-GBM & GOODPASTURE SYNDROME Flashcards
What is the triad of Goodpasture syndrome?
- PROLIFERATIVE CRESCENTIC GN
- PULMONARY HEMORRHAGE
- ANTI-GBM ANTIBODIES
What is highly specific & sensitive for the diagnosis of anti-GBM disease?
- RADIOASSAY ELISA
- IMMUNOBLOTTING FOR ANTIBODIES
Anti-GBM autoantibodies react with epitopes on what type of collagen?
noncollagenous domain of the α3 and α5 chains of type 4 collagen
The antigenic epitope has been localized in the terminal region of what chain of type 4 collagen?
α3-chain
Which 2 sites is the alpha 3 chain of type 4 collagen predominantly found?
- GBM
- ALVEOLAR CAPILLARY BM
Pulmonary disease with hemoptysis have been related to exposure to 6 risk factors?
- HYDROCARBON FUMES
- CIGARETTE SMOKING
- HAIR DYES
- METALLIC DUST
- D-PENICILLAMINE
- COCAINE INHALATION
What virus is associated with Goddpasture syndrome?
INFLUENZA A2
What are the 2 peaks in Anti-GBM disease?
1st peak:
• younger males
• pulmonary hemorrhage
2nd peak:
• older females
• glomerulonephritis
AntiGBM disease limited to the kidney may be more common in what patient population?
OLDER PATIENTS
What precedes the onset of disease in Goodpasture syndrome?
UPPER RESPIRATORY INFECTION
What is the most common biopsy picture in LM of anti-GBM disease?
diffuse crescentic glomerulonephritis involving > 50% of glomeruli, with exuberant, predominantly circumferential crescents
What histopathologic imaging defines the disease process in Goodpasture syndrome and differentiate it from both pauci-immune– and immune complex–mediated forms of crescentic glomerulonephritis?
IF
The diagnostic finding in IF of Goodpasture syndrome is an intense and diffuse linear staining for what type of Ig?
IgG (IgG1, IgG4)
What is the type of staining in IF of Anti-GBM disease?
LINEAR
What are the 3 standard combination therapy used in anti-GBM disease?
- CORTICOSTEROIDS
- CYCLOPHOSPHAMIDE
- PLASMAPHERESIS
