Clinical manifestations, treatment and prognosis analysis of childhood ANCA-associated vasculitis

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CER19662
Paediatric Rheumatology

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Received: 02/01/2026
Accepted : 08/05/2026
In Press: 14/09/2026

Abstract

OBJECTIVES:
To analyse the clinical manifestations and treatment of childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and to identify risk factors for progression to end-stage renal disease (ESRD).
METHODS:
We retrospectively enrolled patients aged <18 years diagnosed with AAV at the First Affiliated Hospital of Zhengzhou University. Comparative analyses were performed between microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) groups regarding clinical manifestations, laboratory findings, disease activity, treatment regimens, and progression to ESRD. Statistical methods included the t-test, chi-square test, Cox regression analysis, and Kaplan-Meier curves.
RESULTS:
A total of 29 patients were included, of whom 22 were female and 23 had MPA. The median age at diagnosis was 13.9 years (IQR 12.3–16.5 years), and the median diagnostic interval was 1 month (IQR 0.6–1.5 months). The most common clinical manifestations were renal, respiratory, and musculoskeletal involvement, and the presentations were similar between MPA and GPA. Fifteen patients progressed to ESRD, 12 of whom had ESRD at initial diagnosis. Glucocorticoids served as the mainstay of treatment. At 12 months post-treatment, 12 patients achieved remission, 3 achieved renal remission, 8 remained on dialysis, and 6 underwent renal transplantation. Long-term survival was achieved in most patients, with one death. Multivariate Cox regression analysis identified elevated serum creatinine as an independent risk factor for progression to ESRD.
CONCLUSIONS:
In this cohort, childhood-onset AAV occurred predominantly in females, with MPA as the major subtype and the kidneys being the most frequently involved organ. Glucocorticoids formed the mainstay of treatment. Approximately half of the children achieved remission following treatment. Patients with elevated serum creatinine levels are at higher risk of progressing to ESRD. Most children achieved long-term survival.