anti-glomerular basement membrane [GBM] disease
Jump to navigation
Jump to search
Etiology
- Goodpasture's syndrome
- influenza virus
- hydrocarbon exposure
- penicillamine
- azathioprine
- connective tissue diseases
- idiopathic (autoimmune)
Epidemiology
- lung & renal involvement (Goodpasture's) typically affects young men[3]
- renal involvement alone generally occurs in older women
Pathology
- anti-glomerular basement membrane (GBM) antibody with specificity for collagen 4 alpha-3 COL4A3
- linear deposition of IgG & complement along glomerular basement membrane
Clinical manifestations
- lung & renal involvement (Goodpasture's) or renal involvement alone
- glomerulonephritis
- pulmonary hemorrhage
- often presents as rapidly progressive glomerulonephritis with pulmonary hemorrhage in > 50% of cases[3]
Laboratory
- urinalysis
- serum chemistries
- creatinine > 6.6 mg/dL predicts poor response to therapy
- urea nitrogen
- anti GBM antibodies in serum
- serum complement levels are normal
- renal biopsy: linear deposition of antibody on immunofluorescence along basement membrane
Complications
Management
- goals
- clearance of anti GBM Ab from serum
- suppression of new anti GBM Ab formation
- initial therapy
- intravenous methylprednisolone, cyclophosphamide & plasmapheresis for pulmonary renal syndrome[6]
- pulse glucocorticoids[3]
- plasmapheresis QD for 2 weeks for ANCA-associated glomerulonephritis associated with pulmonary hemorrhage[3]
- follow with immunosuppressive therapy[3]
- cyclophosphamide 2 mg/kg PO QD for 8 weeks, plus
- prednisone 60 mg PO QD tapered over 8 weeks
- Monitor serum anti GBM antibody titers
- continue immunosuppressive therapy until anti GBM Ab undetectable
- relapse in common, especially in 1st few months
More general terms
More specific terms
Additional terms
- acute leukocytoclastic vasculitis (Henoch-Schonlein purpura; IgA vasculitis)
- anti-glomerular basement membrane (GBM) antibody
- classic polyarteritis nodosa (PAN)
- systemic lupus erythematosus
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 269
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 784
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2015, 2018, 2021
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003 Oct;64(4):1535-50. PMID: https://www.ncbi.nlm.nih.gov/pubmed/12969182 Free Article
- ↑ ARUP Consult: Antiglomerular Basement Membrane Disease - Goodpasture Syndrome The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/antiglomerular-basement-membrane-disease
- ↑ 6.0 6.1 Cite error: Invalid
<ref>tag; no text was provided for refs namedref6
Cite error: <ref> tag with name "ref6" defined in <references> group "" has no content.
Patient information
anti-glomerular basement membrane disease patient information