Anti-glomerular basement membrane disease
Potential etiologies include an unknown environmental trigger, pulmonary infection / injury, and genetic predisposition, although most cases are considered idiopathic. Patients with HLA-DR15 and HLA-DR4 are more susceptible to anti-GBM disease. Occasionally, anti-GBM disease can occur after pulmonary infections. The diagnosis of anti-GBM disease is made in both children and adults. Younger patients are more likely to develop pulmonary hemorrhage.
Anti-GBM disease typically presents with acute renal failure with a nephritic urine sediment. Some patients may have mild renal insufficiency. Lung involvement presents with shortness of breath, cough, hemoptysis, and an abnormal chest x-ray. The definitive diagnosis is made with renal biopsy. Light microscopy of the biopsy specimen will show crescentic glomerulonephritis, while immunofluorescence shows linear deposition of IgG along the glomerular capillaries. Serologic testing can be done to detect anti-GBM antibodies as well.
For more information on Goodpasture syndrome, see OMIM.
M31.0 – Hypersensitivity angiitis
62853008 – Anti-glomerular basement membrane tubulointerstitial nephritis
- Systemic vasculitis
- Diabetic nephropathy
- Lupus nephritis (see systemic lupus erythematosus)
- Acute interstitial nephritis
- IgA nephropathy
- Immune complex glomerulonephritis
- Pauci-immune glomerulonephritis
- Nephrotic syndrome
- Granulomatosis with polyangiitis
- Microscopic polyangiitis
- Alport syndrome
Last Updated: 09/12/2018