Tóm tắt:

Immunoglobulin G4 related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder, marked by tumor-like mass-forming lesions. IgG4-RD is a recently recognized condition and has a few conducted research on the pediatric population. This study aims to provide some insights into the epidemiology, histological findings, diagnosis, and treatment of this condition in children. A systematic literature search was performed on Embase, PubMed, and Web-of-science for case reports and case series of IgG4-RD in children published between 1/2015 and 12/2023. 58 case reports and case series including 62 cases of IgG4-RD in children were identified. The mean age was 12 ± 4.4 years old, of which 54.8% were male. The orbit and central nervous system were two organs predominantly affected. Multi-organ involvement was reported in 14 cases (22.6%). Of 53 patients with serum IgG4 concentration reported, 60.4% of them had elevated serum IgG4 level. 21 patients (33.8%) had fulfilled the definitive diagnosis of the 2020 revised comprehensive diagnostic criteria. Prednisone was the first choice of treatment in 81.8% of the cases. 77.6% of the patients were treated with steroid-sparing DMARDs.