goodpature syndrome anti GBM
- related: Rheumatology
- tags: #literature
Demographics
- mostly white patients in 20s-30s and older women in 60s
Pathogenesis
- small vessel vasculitis
- autoantibody against basement membrane of glomerulus and alveoli
Symptoms
- usually present with pulmonary hemorrhage and AKI with hematuria, proteinuria
- renal more common than pulmonary
- RPGN: rapidly progressive glomerulonephritis and renal failure
- goodpasture differs from GPA with lack of constitutional symptoms
Diagnosis
- Up to 1/3 can have ANCA (usually MPO) antibodies
- diagnosis is suggested by positive anti-GBM antibody
- renal biopsy shows linear IgG immunofluorescence
- goodpasture anti GBM bronchoscopy shows DAH with hemosiderin laden macophages
Treatment
- steroids, cyclophosphamide
- plasmaphresis is unique treatment to anti GBM
More Readings
Links to this note
- Rheumatology
- plasmaphresis is unique treatment to anti GBM
- related: goodpature syndrome anti GBM
- goodpasture anti GBM bronchoscopy shows DAH with hemosiderin laden macophages
- related: goodpature syndrome anti GBM
- goodpasture differs from GPA with lack of constitutional symptoms
- related: goodpature syndrome anti GBM