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ObjectiveWe aimed to validate and modify the renal risk score for antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (AAGN) in a Chinese cohort with a majority of myeloperoxidase (MPO)-positive patients.MethodsA total of 285 AAGN patients with biopsy-proven in our center were retrospectively included. Patients were randomly assigned to the development set (n=201) and the validation set (n=84). We calculated the renal risk score and analyzed the clinicopathological characteristics and follow-up data. The nomogram was constructed based on the independent prognostic factors identified by the multivariable Cox regression and then compared with the renal risk score.ResultsOver a median follow-up period of 41.3 (20.0-63.8) months, 84 (29.5%) patients reached endstage kidney disease (ESKD). In the development set, hypertension (HR=2.163, 95%CI 1.083-4.322, P=0.029), high serum creatinine (HR=1.002, 95%CI 1.001-1.003, P<0.001), high daily urine protein (HR=1.343, 95%CI 1.148-1.571, P<0.001), high glomerular sclerosis (HR=13.983, 95%CI 3.496-55.923, P<0.001), and interstitial fibrosis>50% (HR=4.179, 95%CI 1.900-9.192, P<0.001) were independent risk factors for ESKD, and these indicators were included in the nomogram. The C-indices of the nomogram model in the development set, validation set, and all-data set were 0.838 (0.785-0.891), 0.794 (0.774- 0.814), and 0.822 (0.775-0.869), respectively, which were higher than those of the renal risk score model, 0.801 (0.748-0.854), 0.746 (0.654-0.838) and 0.783 (0.736-0.830), respectively. And the net reclassification improvement and the integrated discrimination improvement further illustrated the higher predictive ability of the nomogram.ConclusionWe present a nomogram as a practical tool to predict renal outcomes in Chinese patients with an MPO-ANCA glomerulonephritis.

Background: Tubulointerstitial involvement has been reported to have a decisive influence on the progression of IgA nephropathy (IgAN). High levels of urine β2-microglobulin (β2-MG) and retinol-binding protein (RBP) were observed in patients with IgAN with tubulointerstitial lesions. However, their roles in disease progression remain unclear. This study aimed to evaluate the associations of urine β2-MG and RBP with the progression of IgAN.Methods: We retrospectively investigated a cohort of 2,153 patients with IgAN. Clinical and pathological features, outcomes, and urine β2-MG, and RBP at the time of biopsy were collected. The associations, of urine β2-MG and RBP with the composite renal outcome, defined as a decline in estimated glomerular filtration rate (eGFR) of ≥50% from baseline or end-stage renal disease (ESRD), were examined using restricted cubic splines and the Cox proportional hazards models.Results: During a median follow-up of 20.40 months, 140 (6.50%) patients reached the composite renal outcomes. Restricted cubic splines showed that patients with higher urinary β2-MG and RBP levels had worse renal outcomes. The Cox regression analysis revealed that urine β2-MG and RBP were associated with a risk of the composite renal outcome in the multivariate adjusted model [+1 SD for log β2-MG, hazard ratio (HR) = 1.462, 95% CI: 1.136–1.882, p = 0.003; +1 SD for log RBP, HR = 1.972, 95% CI: 1.486–2.617, p = 0.001]. The associations were detectable within patients with baseline eGFR &lt;90 ml/min/1.73 m2 (+1 SD for log β2-MG, HR = 1.657, 95% CI: 1.260–2.180, p &lt; 0.001; +1 SD for log RBP, HR = 1.618, 95% CI: 1.199–2.183, p = 0.002), but not among patients with eGFR ≥90 ml/min/1.73 m2.Conclusion: Higher levels of urine β2-MG and RBP were independent risk factors for renal disease progression in IgAN.

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