Glomerulonephritis mediated by antibody to glomerular basement membrane. Immunological, clinical, and histopathological characteristics.

McPhaul, Jr Jj; Jd, Mullins

doi:10.1172/jci108286

Cited by 59 publications

(23 citation statements)

References 27 publications

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“…For example, incidence figures from the literature are variable. Table 3 summarizes the results of prior studies that have described the incidence of anti-GBM disease (8)(9)(10)(11)(12)(13)(14)(15). Although there may be natural variation in disease rates among the populations studied, obvious differences in study methods may account for the different figures reported.…”

Section: Discussionmentioning

confidence: 99%

“…Although there may be natural variation in disease rates among the populations studied, obvious differences in study methods may account for the different figures reported. In half of these studies, an incidence rate could not be calculated because the population at risk was not specified (12,13,15) or could only be estimated (11). Studies of renal biopsy registries have reported comparatively lower incidence rates of approximately 0.6 pmp per year (10,14).…”

Section: Discussionmentioning

confidence: 99%

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Spatial and Temporal Clustering of Anti-Glomerular Basement Membrane Disease

Canney¹,

O’Hara²,

McEvoy³

et al. 2016

CJASN

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Background and objectives An environmental trigger has been proposed as an inciting factor in the development of anti-GBM disease. This multicenter, observational study sought to define the national incidence of anti-GBM disease during an 11-year period (2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014) in Ireland, investigate clustering of cases in time and space, and assess the effect of spatial variability in incidence on outcome.Design, setting, participants, & measurements We ascertained cases by screening immunology laboratories for instances of positivity for anti-GBM antibody and the national renal histopathology registry for biopsy-proven cases. The population at risk was defined from national census data. We used a variable-window scan statistic to detect temporal clustering. A Bayesian spatial model was used to calculate standardized incidence ratios (SIRs) for each of the 26 counties.Results Seventy-nine cases were included. National incidence was 1.64 (95% confidence interval [95% CI], 0.82 to 3.35) per million population per year. A temporal cluster (n=10) was identified during a 3-month period; six cases were resident in four rural counties in the southeast. Spatial analysis revealed wide regional variation in SIRs and a cluster (n=7) in the northwest (SIR, 1.71; 95% CI, 1.02 to 3.06). There were 29 deaths and 57 cases of ESRD during a mean follow-up of 2.9 years. Greater distance from diagnosis site to treating center, stratified by median distance traveled, did not significantly affect patient (hazard ratio, 1.80; 95% CI, 0.87 to 3.77) or renal (hazard ratio, 0.76; 95% CI, 0.40 to 1.13) survival.Conclusions To our knowledge, this is the first study to report national incidence rates of anti-GBM disease and formally investigate patterns of incidence. Clustering of cases in time and space supports the hypothesis of an environmental trigger for disease onset. The substantial variability in regional incidence highlights the need for comprehensive country-wide studies to improve our understanding of the etiology of anti-GBM disease.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Spatial and Temporal Clustering of Anti-Glomerular Basement Membrane Disease

Canney¹,

O’Hara²,

McEvoy³

et al. 2016

CJASN

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“…It reported a disease rate of 1.64 per million population/yr, higher than previous estimates. The disease is well recognized in other white and in Asian populations (9)(10)(11)(12), although is thought to be rarer in African populations (13).…”

Section: Nomenclature and Historymentioning

confidence: 99%

Anti-Glomerular Basement Membrane Disease

McAdoo¹,

Pusey²

2017

CJASN

314

303

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Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized 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 suggests 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 GN, and 40%-60% will have concurrent alveolar hemorrhage. 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, although 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, although de novo anti-GBM disease after transplantation for Alport syndrome is a recognized phenomenon. Copresentation with other kidney diseases such as ANCAassociated 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.

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“…Direct IF tests oftissue were done using incident-light fluorescence microscopy and conventional controls (23,28). For indirect IF using sera and eluates, samples were tested at known Ig concentrations.…”

Section: Methodsmentioning

confidence: 99%