Anti-glomerular basement membrane disease
File(s)
Accepted version
Author(s)
McAdoo, SP
Pusey, CD
Type
Journal Article
Abstract
Anti-glomerular basement membrane (GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypical autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well-characterised autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggest that environmental factors, including infection, may trigger disease in genetically susceptible individuals. The majority of patients develop widespread glomerular crescent formation, presenting with features of rapidly progressive glomerulonephritis, and 40-60% will have concurrent alveolar haemorrhage. Treatment aims to rapidly remove pathogenic autoantibody, typically with the use of plasma exchange, along with steroids and cytotoxic therapy to prevent ongoing autoantibody production and tissue inflammation. Retrospective cohort studies suggest that when this combination of treatment is started early, the majority of patients will have good renal outcome, though presentation with oligoanuria, a high proportion of glomerular crescents, or kidney failure requiring dialysis augur badly for renal prognosis. Relapse and recurrent disease after kidney transplantation are both uncommon, though de novo anti-GBM disease after transplantation for Alport syndrome is a recognised phenomenon. Co-presentation with other kidney diseases such as ANCA-associated vasculitis and membranous nephropathy seems to occur at a higher frequency than would be expected by chance alone, and in addition atypical presentations of anti-GBM disease are increasingly reported. These observations highlight the need for future work to further delineate the immunopathogenic mechanisms of anti-GBM disease, and how to better refine and improve treatments, particularly for patients presenting with adverse prognostic factors.
Date Issued
2017-07-07
Date Acceptance
2017-04-04
Citation
Clinical Journal of the American Society of Nephrology, 2017, 12 (7), pp.1162-1172
ISSN
1555-905X
Publisher
American Society of Nephrology
Start Page
1162
End Page
1172
Journal / Book Title
Clinical Journal of the American Society of Nephrology
Volume
12
Issue
7
Copyright Statement
Copyright © 2017 by the American Society of Nephrology
Sponsor
Imperial College Healthcare NHS Trust- BRC Funding
Grant Number
RDA04 79560
Subjects
1103 Clinical Sciences
Urology & Nephrology
Publication Status
Published
Date Publish Online
2017-07-07