Anti-Glomerular Basement Membrane Disease
File(s)CJASN Anti-GBM Review V2 CLEAN.docx (2.58 MB)
Accepted version
Author(s)
Gulati, K
McAdoo, SP
Type
Journal Article
Abstract
Anti–glomerular basement membrane (GBM) disease often presents with rapidly progressive glomerulonephritis with or without lung hemorrhage. It is rare to present with lung hemorrhage alone. • Antibodies are generally directed at EA or EB epitopes found within the noncollagenous (NC1) domain of the α3 chain of type IV collagen. • Renal and patient survival in untreated anti-GBM is poor, although intensive therapy provides improved outcomes in patients with alveolar hemorrhage or rapidly progressive glomerulonephritis not requiring dialysis at outset. • Relapses are uncommon in patients with anti-GBM disease. • Patients who are double positive for antineutrophil cytoplasm antibody and anti-GBM have more relapsing disease and often need maintenance immunosuppressive therapy.
Date Issued
2018-11-01
Date Acceptance
2018-09-01
ISSN
0889-857X
Publisher
Elsevier
Start Page
651
End Page
673
Journal / Book Title
Rheumatic Disease Clinics of North America
Volume
44
Issue
4
Copyright Statement
© 2018 Elsevier Inc. All rights reserved. This manuscript is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International Licence http://creativecommons.org/licenses/by-nc-nd/4.0/
Sponsor
Imperial College Healthcare NHS Trust- BRC Funding
Identifier
http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000449038400011&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=1ba7043ffcc86c417c072aa74d649202
Grant Number
RDA04 79560
Subjects
Science & Technology
Life Sciences & Biomedicine
Rheumatology
Goodpasture syndrome
Vasculitis
Rapidly progressive glomerulonephritis
Pulmonary hemorrhage
Plasma exchange
ANTI-GBM DISEASE
RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS
ANTINEUTROPHIL CYTOPLASMIC ANTIBODY
PULMONARY-RENAL SYNDROME
T-CELLS
GOODPASTURES-SYNDROME
CRESCENTIC GLOMERULONEPHRITIS
MULTIPLE-SCLEROSIS
PROGNOSTIC-SIGNIFICANCE
ALEMTUZUMAB TREATMENT
1103 Clinical Sciences
Arthritis & Rheumatology
Publication Status
Published
Date Publish Online
2018-09-07