Comparison of IgG4 related disease in patients aged ≤ 25 years (pediatric, adolescent, and young adult) and those aged ≥ 50 years

Abstract

Objectives

To evaluate and compare the manifestations of IgG4-RD in patients ≤ 25 years of age (pediatric, adolescents and young adults) and those ≥ 50 years of age.

Materials and methods

A retrospective descriptive study of 64 patients diagnosed with IgG4-RD based on the Umehara 2011/2020 criteria and the ACR/EULAR 2019 classification criteria, covering the period from 2014 to 2023. The medical records of these two age groups were reviewed.

Results

Of the 64 patients, 7 were ≤25 years old (11%) and 35 were ≥50 years old (54.7%). Among patients ≤ 25 years old, 6 were female, with a mean age at consultation of 21.3 years (± 5.06). Five (71%) had single-organ involvement, while two presented with multi-organ involvement. In the ≥50 years group, females also predominated (57%), with a mean age at consultation of 60 years (± 7.4) and more constitutional symptoms were observed, multi-organ involvement (p = 0.009), asthenia (p = 0.047), lymph nodes (p = 0.047), submandibular gland involvement (p = 0.009), lung involvement (p = 0.013) and parotidomegaly (p = 0.097). Ocular involvement was more frequent in those under 25 years of age; however, in those over 50 years of age, this involvement was mostly bilateral (p = 0.011). There were no statistically significant differences in laboratory variables between both groups. There was a predominance of lymphoplasmacytic infiltrate in the majority of the biopsies performed. In cases of ocular involvement, histopathological analysis revealed lymphoplasmacytic infiltrate with no differences in the presence of storiform fibrosis, obliterative phlebitis, or eosinophilic infiltrate. However, the latter was more frequent in older patients.

Conclusion

Reviewing the literature and describing our data on IgG4-RD in children is crucial, as pediatric physicians often overlook this condition. This fact, as in adult cases, could help prevent unnecessary surgeries and irreversible organ damage.