Childhood Sjogren's syndrome: An Italian case series and a literature review-based cohort.

OBJECTIVE:Sjogren's syndrome (SS) is a chronic autoimmune disease with a highly variable presentation. This study aims to describe childhood SS (cSS) features to help guide clinicians in their consideration of and workup for cSS. METHODS:We retrospectively reviewed medical records of patients with cSS referred to three Italian pediatric rheumatology centers from 2015 to 2019 and we conducted a literature review of cSS. Statistical analysis was performed to detect associations between clinical/laboratory features. RESULTS:We reviewed 12 cases (9 female) followed in 3 Italian centers and 240 cases (191 female) in the published literature reporting individual information. The median age at disease onset was 10 years for both cohorts. The most frequently reported clinical SS-specific feature was parotitis in both cohorts (67% each). Extraglandular manifestations were very common and joint involvement was the most frequent. In the cluster analysis, we identified a significant association between parotitis and younger patients (< 11 years). We verified the presence of the main SS features (exocrine gland inflammation, exocrine gland dysfunction, and presence of autoantibodies) in the Italian cohort and the literature review-based cohort: 92% and 80% of the cohorts, respectively, had at least 2/3 main characteristics. CONCLUSION:We described cSS features with relative frequencies and we found that parotid involvement was related to cSS in younger patients. The majority of patients showed various combinations of exocrine gland inflammation, exocrine gland dysfunction, and presence of autoantibodies giving a theoretical basis for future research to pave the way for the development of cSS specific diagnostic criteria.