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Anti-glomerular basement membrane disease
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Anti-glomerular basement membrane disease

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Contributors: Amirah Khan MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Anti-glomerular basement membrane disease (anti-GBM) is an autoimmune disorder characterized by rapidly progressive glomerulonephritis and possible pulmonary hemorrhage. The formation of antibodies against the pulmonary alveoli and the basement membrane of the glomerulus is short lived, and symptoms may not be noticeable for several months afterwards. Pulmonary involvement is observed in 40%-60% of patients. The spectrum of both renal and pulmonary disease (or more rarely of renal disease alone) caused by anti-GBM antibodies is known as Goodpasture syndrome. This disease is rare.

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.

Codes

ICD10CM:
M31.0 – Hypersensitivity angiitis

SNOMEDCT:
62853008 – Anti-glomerular basement membrane tubulointerstitial nephritis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 07/25/2018
Last Updated: 09/12/2018
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Anti-glomerular basement membrane disease
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Anti-glomerular basement membrane disease : Cough, Creatinine elevated, Microscopic hematuria, Proteinuria, Dyspnea, Hemoptysis, BUN elevated
Copyright © 2019 VisualDx®. All rights reserved.