Abstract
Background: To evaluate the diagnostic accuracy of antineutrophil cytoplasmic antibody (ANCA) renal risk score (ARRS) for prediction of renal outcome in patients with ANCA-associated glomerulonephritis (ANCA-GN).Methods: We searched PubMed, EMBASE, Ovid, Web of Science, the Cochrane Library, and ClinicalTrials.gov for studies, which used ARRS to predict end-stage renal disease (ESRD) in patients with ANCA-GN. Two reviewers independently screened articles for inclusion, assessed the quality of studies with both an adapted Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool. We calculated the combined patients with ESRD in the ARRS categories and presented the summary and individual estimates based on the ARRS categories. Then, the sensitivity, specificity, diagnostic odds ratio (DOR), positive/negative likelihood ratio, and the area under the receiver operating characteristic (AUROC) curves of the pooled data for ARRS were used to assess the accuracy of the “above the low-risk threshold” (ARRS ≥ 2) and “high-risk grade” (ARRS ≥ 8) for renal outcome of patients with ANCA-GN. The hierarchical summary ROC (HSROC) was used to verify the accuracy value. The clinical utility of ARRS was evaluated by the Fagan plot. Heterogeneity was explored using meta-regression and subgroup analysis.Results: A total of 12 distinct cohorts from 11 articles involving 1,568 patients with ANCA-GN were analyzed. The cumulative patients with ESRD at the maximum follow-up of 60 months was 5% (95% CI: 0.02–0.07; p < 0.001) for ANCA-GN with low ARRS (0–1 points) and significantly increased to 22% (95% CI: 0.15–0.29; p < 0.001) medium ARRS (2–7 points). The combined cumulative patients with ESRD was 59% (95% CI: 0.49–0.69; p < 0.001) high ARRS (8–11 points). The pooled sensitivity of ARRS ≥ 2 in predicting ESRD was 98% with a specificity of 30% and a DOR of 15.08 and the mean AUROC value was 0.82. The pooled sensitivity of ARRS ≥ 8 in predicting ESRD was 58% with a specificity of 86% and a DOR of 7.59. The meta-regression and subgroup analysis indicated that variation in the geographic regions, study design, index risk, follow-up time, age of patient, publication year, and number of patient could be the potential sources of heterogeneity in the diagnosis of ARRS ≥ 8.Conclusion: This meta-analysis emphasized the good performance of the ARRS score in predicting the renal outcome in patients with ANCA-GN. However, these findings should be verified by future large-scale prospective studies.
Highlights
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of life-threatening systemic autoimmune diseases characterized by inflammation, which included granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatous polyangiitis (EGPA), and renal-limited vasculitis (RLV) [1]
After excluding the articles with irrelevant contents and articles with no full-text and insufficient data, we included 12 distinct cohorts from 11 articles [11, 19,20,21,22,23,24,25,26,27,28] in the meta-analysis. These 12 studies involving 1,568 patients with ANCA-associated glomerulonephritis (ANCA-GN) were performed in different geographic regions including Europe (n = 6), Asia (n = 3), North America (n = 1), Africa (n = 1), and multicenter (n = 1)
We identified 12 published cohort studies that reported the cumulative patients with end-stage renal disease (ESRD) in three ANCA renal risk score (ARRS) grades
Summary
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of life-threatening systemic autoimmune diseases characterized by inflammation, which included granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatous polyangiitis (EGPA), and renal-limited vasculitis (RLV) [1]. In 2010, an international working group of renal pathologists published a histological classification for ANCA-GN based on kidney biopsy. This classification divided patients to four subgroups: focal (>50% normal glomeruli), crescentic (>50% cellular crescents), sclerotic (>50% sclerotic glomeruli), and mixed (any other combination) and the probability of progressing to ESRD increased in ascending order of focal, crescentic, mixed, and sclerotic [7]. To evaluate the diagnostic accuracy of antineutrophil cytoplasmic antibody (ANCA) renal risk score (ARRS) for prediction of renal outcome in patients with ANCA-associated glomerulonephritis (ANCA-GN)
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