Research Information System
31st European Paediatric Rheumatology Congress, Gothenburg, Sweden, 11 - 14 September 2024, pp.7-8, (Full Text)
Introduction: IgG4-related disease (IgG4-RD) is a fbroinfammatory disease, afecting almost any organ. Given its rarity and the paucity of pediatric clinical trials, our knowledge of the diagnosis and management is mainly based on adult experience and reports of case series in children.
Objectives: We aimed to report the characteristics of pediatric IgG4-RD through a multicenter registry, to assess disease clusters, and to evaluate the performances of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classifcation criteria and the 2020 revised comprehensive diagnostic (RCD) criteria in this cohort.
Methods: Data of IgG4-RD patients in 13 pediatric rheumatology centers were recorded to a web-based registration system. The diag nosis of IgG4-RD was made according to the 2011 comprehensive diagnostic criteria. The clinical phenotypes, disease subsets, and sensitivities of the criteria sets were also investigated.
Results: Thirty-fve children (19 females and 16 males) with IgG4-RD were enrolled. The median age at diagnosis and median follow-up
time were 13.3 (25p-75p; 9.9-15.2) years and 1.8 (25p-75p; 0.6-3.4) years, respectively. The most common organ involvement was ocu-
lar fndings in 21 (60%) patients, followed by lymph nodes in 12 (34.3%), musculoskeletal system in 12 (34.3%), neurological system
in 9 (25.7%), pancreatic and biliary tract in 8 (22.9%) and lacrimal and major salivary glands in 8 (22.9%) patients. Sixteen patients (45.7%) were classifed as having proliferative subtype whereas 19 (54.3%) had fbrotic subtype. We identifed three clusters in our study cohort: those with eye involvement (n=11, 31.4%), those with eye involvement and neurological fndings (n=15, 42.9%), and those with pancreato-hepatobiliary disease and lymph node involvement (n=9, 25.7%). Serum IgG4 levels were high in 19 out of 28 patients (67.8%). Biopsy was performed in 31 patients (88.6%) in diagnostic process and 27 of them (87.0%) had fndings associated with IgG4-RD. Dense lymphocytic infltrate and storiform fbrosis were the most prominent histopathological fndings. The sensitivities of the 2019 ACR/EULAR classifcation criteria and the 2020 RCD criteria were 5.7% and 88.5%, respectively. All patients except one received corticosteroid treatment, and azathioprine was the most preferred drug as a steroid-sparing agent. Complete response was observed in 33.3% of the patients, partial response in 23.3% and stable disease in 6.6%. Relapse occurred in 11 patients (31.4%).
Conclusion: IgG4-RD has a wide variety of clinical manifestations, however in children the most common presentation was orbital
involvement. The 2020 RCD criteria had a better performance whereas the 2019 ACR/EULAR classifcation criteria performed poorly in pediatric patients.