Etiology, clinical profile, and outcomes of crescentic glomerulonephritis in children: a systematic review

Abstract

Background: Crescentic glomerulonephritis, if not managed promptly, is associated with unsatisfactory outcomes. There are limited studies reporting the outcomes of crescentic glomerulonephritis in children.

Objectives: This systematic review is aimed at synthesizing the data on etiology, clinical profile, and outcomes of crescentic glomerulonephritis in children.

Data sources: We performed a literature search using PubMed, Embase, and Web of Science from inception to January 2024 without language or geographic restrictions.

Study eligibility criteria: Cohort and cross-sectional studies with at least 10 participants reporting etiology, clinical features, and outcomes on crescentic glomerulonephritis in children were considered eligible.

Participants and interventions: Children aged less than 18 years with crescentic glomerulonephritis.

Study appraisal and synthesis methods: We used a tool by Hoy et al. for assessing the quality of studies. We calculated pooled estimates using random effect meta-analyses. Primary outcome was the pooled proportion of patients progressing to kidney failure.

Results: From 1706 records, we included 36 studies (1548 participants) from 16 countries. Etiology was immune-complex glomerulonephritis in 76% (95% CI 67 to 85), pauci-immune in 19% (13 to 25), and anti-GBM disease in 5% (3 to 7) of patients. Gross hematuria, oliguria, edema, and hypertension were observed in 63% (41 to 82), 57% (34 to 79), 79% (65 to 90), and 64% (49 to 77), respectively. In-hospital mortality, reported in 11 studies, was 7% (4 to 11). Progression to kidney failure and chronic kidney disease was reported in 27% (21 to 33) and 50% (29 to 71) of patients, respectively. Risk factors for kidney failure included oliguria, dialysis requirement at onset, estimated GFR, proportion of fibrous crescents, and pauci-immune glomerulonephritis as the underlying etiology.

Limitations: High heterogeneity in pooled estimates of the outcomes.

Conclusions and implications of key findings: Immune-complex glomerulonephritis is the most common etiology in children, with edema, hypertension, gross hematuria, and oliguria being the chief presenting manifestations. Almost one in every four patients with crescentic glomerulonephritis progressed to kidney failure.

Systematic review registration number: PROSPERO registration number CRD42024500515.

Keywords: Chronic kidney disease; Crescents; Kidney failure; Rapidly progressive glomerulonephritis.