Achille Marino1, Micol Romano2, Teresa Giani3, Carla Gaggiano4, Stefania Costi5, Revika Singh6, Jay J Mehta7, Scott M Lieberman8, Rolando Cimaz9. 1. Department of Pediatrics, Desio Hospital, ASST Monza. Via Mazzini 1, 20832 Desio (MB), Italy; ASST G.Pini-CTO, Via Gaetano Pini 9, 20122 Milan, Italy. Electronic address: achillemarino6@gmail.com. 2. ASST G.Pini-CTO, Via Gaetano Pini 9, 20122 Milan, Italy. 3. Pediatric Rheumatology, Meyer Children's Hospital, Viale Gaetano Pieraccini, 24, 50139 Florence, Italy; Department of Medical Biotechnology, University of Siena, viale Mario Bracci, 16, Siena, Italy. 4. Department of Pediatrics, University of Siena, viale Mario Bracci, 16, Siena, Italy. 5. University of Milan, Via Gaetano Pini 9, 20122 Milan. 6. Northwestern University, 633 Clark St, Evanston, IL 60208, USA. 7. Division of Rheumatology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA 19104-4399, USA. 8. Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa, 200 Hawkins Drive, Iowa City, IA 52242, USA. 9. ASST G.Pini-CTO, Via Gaetano Pini 9, 20122 Milan, Italy; Department of Clinical Sciences and Community Health, and Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Via della Commenda 19, 20122 Milan, Italy.
Abstract
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.
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.
Authors: Rachel L Randell; Sara M Stern; Heather Van Mater; Laura E Schanberg; Scott M Lieberman; Matthew L Basiaga Journal: Pediatr Rheumatol Online J Date: 2022-09-05 Impact factor: 3.413