AUTHOR=Zhang Pei , Yang Xiao , Gao Chun-lin , Wu Wei , Xia Zheng-kun TITLE=Crescentic glomerulonephritis in children: short-term follow-up predicts long-term outcome JOURNAL=Frontiers in Pediatrics VOLUME=11 YEAR=2023 URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2023.1206168 DOI=10.3389/fped.2023.1206168 ISSN=2296-2360 ABSTRACT=Background

Crescentic glomerulonephritis (CrGN) is a relatively rare but severe condition in childhood with the clinical feature of rapidly progressive glomerulonephritis (RPGN). The aim of this study is to investigate the clinicopathological features and prognosis of CrGN in children.

Methods

We retrospectively analyzed the clinical and laboratory data, renal pathological results, treatment, and outcome of 147 CrGN in two Chinese pediatric nephrology centers.

Results

Among the 147 children, there were 22 cases of type I (15.0%), 69 cases of type II (46.9%), and 56 cases of type III (38.1%). The mean percentages of crescents in CrGN I, II, and III were 85.3%, 68.7%, and 73.6%, respectively. The children with type I CrGN presented with more severe clinical manifestations and pathological lesions. The 3-month cumulative renal survival rates of types I, II, and III CrGN were 66.3%, 93.6%, and 75.6%, respectively. The 1-year cumulative renal survival rates of types I, II, and III CrGN were 56.9%, 85.3%, and 73.1%, respectively, and the 5-year cumulative renal survival rates of types I, II, and III CrGN were 33.8%, 73.5%, and 47.1%, respectively. The Kappa Consistency Test between the 3-month and 1-year total renal survival (82.1% vs. 74.7%) of the children was 0.683 (P < 0.001), and between the 1-year and 5-year total renal-free survival (78.3% vs. 69.1%) of the children was 0.476 (P < 0.001). The Bowman's Capsule Rupture (BCR), crescent, interstitial inflammation, and interstitial fibrosis/tubular atrophy (IF/TA) score were predictors of end-stage kidney disease (ESKD) risk but BCR showed better predictive value for ESKD than interstitial inflammation score (P = 0.027) and IF/TA score (P = 0.047).

Conclusion

Patients with type I tended to have the worst renal survival rates. The three-month renal prognosis could partially reflect the 1-year renal prognosis, and the 1-year mortality rate could partially reflect the 5-year mortality rate of children with CrGN.