The Immunopathological Spectrum of Crescentic Glomerulonephritis: A Survey of 106 Patients in a Single Chinese Center

Lin, Wei; Chen, Min; Cui, Zhao; Zhao, Minghui

doi:10.1159/000314665

Cited by 26 publications

(23 citation statements)

References 55 publications

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“…Of these, 6.9% were due to anti-GBM disease, 52.3% to pauciimmune disease and 40.9% to immune complex disease (of whom 14.3% were due to lupus nephritis) [3]. In a recent report from China on 106 patients with crescentic GN, the percent prevalences were 16% for anti-GBM disease, 40.6% for immune complex disease and 43.4% for pauciimmune disease [4].…”

Section: Introductionmentioning

confidence: 99%

Glomerulonephritis with crescents among adult Saudi patients outcome and its predictors

et al. 2011

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The aim of this study is to investigate the clinical and pathological features and outcome of glomerulonephritis with crescents among adult patients. This is a retrospective study of all cases of crescentic GN seen over a 9-year period (2001-2010). Histological features were assessed, and renal function at baseline and end of follow-up period was recorded. Results among different etiological groups at baseline and end of follow-up period were compared. The mean age in the whole group was 35.6 years (16.2), with the lowest mean in the lupus nephritis (LN) group [27.7 years (9.9)] and the highest in the pauciimmune glomerulonephritis (PIGN) group (P = 0.001). There were 72 cases enrolled in the study. LN accounted for 49.3% of the cases, PIGN for 26.5%, other immune complex glomerulonephritis (ICGN) for 19% and post-infectious GN accounted for 6.3% The majority (85.7%) of the patients had renal impairment at presentation (mean serum creatinine levels were 247 (85) μmol/l, 412 (75) μmol/l and 230 (141) μmol/l in LN, PICN and ICGN, respectively (P = 0.05). Women accounted for 85.3, 76.5 and 36.2% of the patients in LN, PICN and ICGN, respectively (P = 0.025). By the end of the follow-up period of 26 (22.9) months, 25.8% of the patients were requiring dialysis (16.70% in the LN group, 50% in PIGN and 25% in ICGN (P = 0.05) and 21.7% had nephrotic range proteinuria (16.7, 1 and 33.3%, respectively (P = 0.4). Using logistic multivariate analysis, the only independent factors found to predict need for dialysis of prognosis were percent of sclerosed glomeruli (P = 0.05) and presence of ATN (P = 0.028). Baseline proteinuria or SCr, gender and number of glomeruli with crescents, on the other hand, did not impact prognosis. Using linear regression multivariate analysis, SCr, protein excretion and activity score at biopsy did not influence change in SCr or final SCr during the follow-up period. Using ANOVA to compare the groups of LN, PIGN and ICGN), we found significant differences only in gender between LN and ICGN (P = 0.035), in percent glomerular global sclerosis (between LN and PIGN (P = 0.007) and between LN and ICGN (P = 0.012) and in age (between LN and PIGN (P = 0.006). Almost half of our patients with CrGN were due to LN which is higher than that reported by others where PIGN was the more prevalent etiology. Patients with PICN were older and had worse prognosis. This could be explained by the higher number of globally sclerosed glomeruli in the PIGN group.

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Section: Introductionmentioning

confidence: 99%

Glomerulonephritis with crescents among adult Saudi patients outcome and its predictors

et al. 2011

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“…The anti-GBM disease incidence (0.5–1 case/million/year) is vastly lower compared to that of other autoimmune kidney diseases, such as antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (10–20 cases/million/year) or lupus nephritis (20–100 cases/million/year) [1]. Although rare, anti-GBM disease is often lethal, as most of the patients present with rapidly progressive glomerulonephritis that accounts for up to 20% of acute renal failure [2,3]. Untreated anti-GBM disease has an almost universally poor outcome with death from renal failure or lung hemorrhage.…”

Section: Introductionmentioning

confidence: 99%

Advances in the Genetics of Anti-Glomerular Basement Membrane Disease

Zhou

Zhao

et al. 2010

Am J Nephrol

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Anti-glomerular basement membrane (GBM) disease is a rare but lethal autoimmune disorder. Over the past few years, the nature of the autoantigen and its epitopes has been defined, as well as the possible pathogenic role played by environmental factors, and by cellular and humoral immunity. However, the majority of data on anti-GBM disease comes from studies conducted on animal models, since human studies are relatively scarce. Genetic studies have highlighted strong positive associations of anti-GBM disease with the HLA-DRB1*1501 allele. In addition, the disease has been associated with genes of the FCGR and KLK families. Important as they are, these findings have to be considered preliminary, if not contentious. Here, we provide an overview of recent discoveries in the genetics of anti-GBM disease that may help elucidate the disease pathogenesis while improving therapeutic approaches. We also discuss the limitations of such discoveries. Finally, we suggest that extensive collaboration between investigators and novel integrative approaches are essential to the progress of our understanding of the anti-GBM disease. We attempted to summarize the current knowledge on the pathogenesis of anti-GBM disease by providing different but complementary perspectives from previous reviews.

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“…The most common etiology of ICGN in the current study was IgA nephropathy followed by lupus and PIGN. A few studies from Asia have reported ICGN as the predominant etiology in adults [10][11][12][13] .…”

Section: Resultsmentioning

confidence: 99%

“…[12] Another interesting observation is the higher proportion of patients with PIGN, a disease that is considered to be common in the paediatric population. [14] The high prevalence of PIGN in the current study might be due to the tropical location and poor socioeconomic conditions predisposing to infections.…”

Section: Resultsmentioning

confidence: 99%

“…[9][10][11] The histological parameters were comparable in the two groups except for the higher proportion of sclerosed glomeruli in ICGN. This might be secondary to the high proportion of patients with IgA nephropathy and lupus nephritis, which follows a more indolent course [12] . Two-third of PauciGN patients showed ANCA positivity, similar to what reported from previous series [8] .…”

Section: Resultsmentioning

confidence: 99%

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