Recurrent immunoglobulin A vasculitis in children and adolescents: prevalence and associated risk factors

Abstract

Background: Immunoglobulin A vasculitis (IgAV) is a systemic vasculitis characterized by potential recurrence. Purpose: This study aimed to explore the prevalence of and factors associated with IgAV recurrence. The clinical phenotypes of childhood-and adolescent-onset IgAV were described and compared. Methods: This retrospective cohort analysis included patients aged ≤18 years and diagnosed with IgAV treated in a tertiary academic center between January 2010 and December 2022. Recurrence was classified by the reappearance of purpuric/petechiae or other features that reappeared after at least 1 asymptomatic month. Results: The 361 patients with IgAV had a mean age of 7.7±3.9 years; 53.2% of them were female. All patients with IgAV exhibited skin manifestations. Gastrointestinal (GI) and musculoskeletal (MSK) features were observed in 214 (59.3%) and 219 patients (60.7%), respectively. Renal involvement was observed in 82 patients (22.7%). The prevalence of childhood-and adolescent-onset IgAV was 269 (74.5%) and 92 (25.5%), respectively. Patients with childhood-onset IgAV had significant GI manifestations (P=0.01), had MSK features (P=0.002), and required hospitalization (P=0.004). In contrast, patients with adolescent-onset IgAV had significant renal involvement (P<0.001) and required a longer duration of corticosteroids treatment (P<0.001). Of the study population, 119 patients (35.6 %) experienced recurrence. During the 12-month follow-up period, 98 patients (27.1%) had recurrent IgAV at a median 1.9 months (interquartile range, 1.2–4.8 months). On the multivariable Cox proportional hazards regression analysis, corticosteroids treatment was significantly associated with IgAV recurrence (hazard ratio, 1.99; 95% confidence interval, 1.266–3.157; P=0.003). Conclusion: Renal involvement was more prevalent in adolescent-onset IgAV, whereas MSK and GI involvement were more common in childhood-onset IgAV. Recurrence was noted in 35.6% of the population. Therefore, vigilant monitoring for recurrence is necessary.