Crescentic IgA Nephropathy

Jg, Abuelo; Ar, Esparza; Ra, Matarese; Rg, Endreny; Js, Carvalho; Allegra,

doi:10.1097/00005792-198411000-00005

Cited by 51 publications

(19 citation statements)

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“…This is comparable to studies reporting all IgA nephropathy and nephropathy associated with HSP (table 1) [8,9,10, 14,26,27,28,29,30,31,32,33,34,35,36]. It is difficult to assess from these studies the proportion of patients who had vasculitic/crescentic lesions as a component of their disease, but in studies where this was stated, outcomes were 60% in ESRF [37] and 3% in ESRF [21] compared to 15% in our series.…”

Section: Discussionsupporting

confidence: 63%

Vasculitic IgA Nephropathy: Prognosis and Outcome

et al. 2009

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Background: A vasculitic glomerulonephritis is seen in both IgA nephropathy and Henoch-Schönlein purpura. No study has examined prognostic factors in vasculitic IgA nephropathy. Methods: All patients with vasculitic IgA presenting to our centre between January 1996 and May 2003 were retrospectively reviewed by analysis of clinical and pathological features at presentation and during follow-up. Results: Of 363 patients with IgA nephropathy, 67 patients had vasculitic IgA nephropathy (33 Henoch-Schönlein purpura; 34 IgA). Median length of follow-up was 32.38 months (range 0–90). Median glomerular filtration rate at presentation was 45.56 ml/min (range 4–110), arterial blood pressure 104.67 mm Hg and proteinuria 1.19 g/24 h (range 0–14.8) falling during follow-up to 0.11 g (range 0–3.1). Median chronic damage at presentation in the biopsy was 10% (range 0–91). The majority (79%) were immunosuppressed with 84% patient survival and 85% renal survival at 60 months. Three factors significantly affected renal outcome: renal function; blood pressure at presentation and the amount of chronic damage in the biopsy. The effect of immunosuppression on outcome was difficult to comment on as treated and untreated groups were not comparable. Conclusions: Presenting renal function, blood pressure and chronic damage in the biopsy are important prognostic factors in vasculitic IgA nephropathy. Immunosuppression is advocated in some patients.

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Section: Discussionsupporting

confidence: 63%

Vasculitic IgA Nephropathy: Prognosis and Outcome

et al. 2009

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“…41 This may explain the increased renal IgM deposition in those with low C4 levels and the negative correlation between serum C4 and renal deposition of IgM in the current study. The gradually accumulated renal IgM deposition is associated with heavy proteinuria, 42 crescents formation, 43,44 increased mesangial matrix as well as mesangial and perimesangial electron-dense deposits 45 leading to a poor outcome in IgAN. 46 We presumed that the lectin pathway may not be activated sufficiently under the condition of C4 deficiency, which protect the glomeruli from the initial injury at the time of renal biopsy.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of IgA nephropathy associated with low complement 4 levels

Zhu

Lin

et al. 2014

Renal Failure

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Objective: C4 deficiency is the most commonly inherited immune disorder in human. The present study investigated the characteristics of the IgAN patients with low serum C4 levels.Methods: We performed a prospective observational study. Clinical as well as histopathologic parameters were assessed. A Kaplan-Meier survival analysis was performed concerning the primary outcome defined as the serum creatinine increased 1.5-fold from baseline. The prognostic significances of clinical and histopathologic parameters were determined using Cox proportional hazards models. Results: Five-hundred twelve biopsy proven IgAN cases were available for analysis with a median follow-up of 38.4 months. Ninety-nine cases (19.34%) presented with low C4 levels (LowC4 group) and the other 413 cases did not (NlowC4 group). At the time of renal biopsy, renal injury was lighter in the LowC4 group compared with the NlowC4 group. Renal C4 deposition was significantly decreased while IgM deposition was increased in the LowC4 group. A correlation analysis shows that lower C4 levels were associated with better renal presentations at biopsy. However, the risk of developing the primary outcome was significantly greater in those with low C4 levels. Specifically, during the follow-up period, the risk of developing primary outcome was nearly ten folds higher in those with low C4, compared to those without low C4. Conclusion: There is a high prevalence of low C4 levels in IgAN patients. These patients with low C4 levels exhibited better renal presentations at the time of renal biopsy, whereas might be associated with a poor prognosis.

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“…The patients with IgA nephropathy occasionally present small crescents in histologic examination [6,16]. Tang et al [17] has observed that there were 20.5% IgA nephropathy patients with a varied degree of crescent formation.…”

Section: Discussionmentioning

confidence: 99%

“…Initial reports suggested that primary IgA nephropathy has a benign course; however, progression to end-stage renal failure was subsequently observed from 8 to 33% [4,5]. Crescentic formation in IgA nephropathy, histologically characterized by extensive extracapillary proliferation, is assumed to have a poor prognosis [6]. Moderate (less than 30%) crescentic involvement has been occasionally reported to recover spontaneously [7], but recently it has been suggested that even small and very focal crescents should be regarded as an indication for unfavorable prognosis [8].…”

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confidence: 99%

“…Moderate (less than 30%) crescentic involvement has been occasionally reported to recover spontaneously [7], but recently it has been suggested that even small and very focal crescents should be regarded as an indication for unfavorable prognosis [8]. Some patients of IgA nephropathy showed a rapidly progressive deterioration of renal function associated with diffuse crescent formation (more than 50%), which was diagnosed as diffuse crescentic IgA nephropathy (DCIgAN) [6,9]. Recently, the increasing incidence of diffuse crescentic glomerulonephritis has been found with a significantly increasing number of annual nontransplanting renal biopsies in our department, and in an 11-year period from 1990 to 2001, 25 patients were diagnosed as DCIgAN [10].…”

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confidence: 99%

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Idiopathic IgA Nephropathy with Diffuse Crescent Formation

Tang

Wang

et al. 2002

Am J Nephrol

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Objective: To investigate the clinicopathological features and outcome of idiopathic IgA nephropathy with diffuse crescent formation in Chinese patients. Methods: Twenty-five patients with diffuse crescentic IgA nephropathy (DCIgAN), 15 males and 10 females with median age of 28.5, and median disease duration of 5.1 months, were studied. Their clinical, laboratory and pathological features and outcome were investigated. Twenty-one were administered pulse immunosuppressive therapy, and 15 were followed up for more than 6 months. Results: 1.14% had total IgA nephropathy, and 16.4% total diffuse crescentic glomerulonephritis. Clinically, most of patients (88%) showed rapidly progressive glomerulonephritis associated with a high level of serum creatinine (418 ± 264 µmol/l). Gross hematuria was noted in 72%, hypertension in 64%, and nephrotic syndrome in 48%. Pathologically, except for diffuse crescent formation (a median 65% and range 50–95%), we observed segmental necrosis of glomerular capillaries in 60%, glomerular infiltrating cells in 48%, endothelial cells proliferation in 32%, and rupture of Bowmans’ capsule in 24%. Severe tubular interstitial damage was also found, tubular atrophy in 64%, interstitial fibrosis in 60%, diffuse interstitial infiltrating cells in 74%, and interstitial vasculitis in 40%. Immunopathologically, four phenotypes were observed; however, IgA associated with IgM deposition was higher than that in patients with general IgA nephropathy (IgAN). In addition, the infiltrating CD4+, CD8+, CD68+ and PCNA+ cells in renal tissue were significantly high compared with that in controls. In a follow-up study, 66.7% of patients had life-sustaining renal function, 4 of them had normal range of serum creatinine (<124 µmol/l), and only 5 were dialysis-dependent. Conclusions: The patients with crescentic IgA nephropathy mostly show rapidly progressive nephritis associated with more severe pathological changes including glomerular, tubular interstitial and vascular lesions than in patients with general IgAN. The infiltrates in glomeruli may contribute to the crescentic formation, and the intensive immune suppressing treatment is useful to improve renal damage in patients with DCIgAN.

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Crescentic IgA Nephropathy

Cited by 51 publications

References 0 publications

Vasculitic IgA Nephropathy: Prognosis and Outcome

Vasculitic IgA Nephropathy: Prognosis and Outcome

Clinical characteristics of IgA nephropathy associated with low complement 4 levels

Idiopathic IgA Nephropathy with Diffuse Crescent Formation

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