Background: Sjögren’s Syndrome (SS) is a chronic systemic autoimmune disease characterized by involvement of the salivary and lacrimal glands. Pediatric SS is rare and has a different presentation compared to adults, with extraglandular manifestations predominating and, less frequently, classic symptoms of sicca syndrome.

Objectives: The objective of this study is to describe the patients registered with a suspected diagnosis of primary/secondary pediatric SS in a reference Pediatric Rheumatology unit, as well as to evaluate the classifying criteria of the ACR/EULAR (2016) and the pediatric diagnostic criteria proposed by Tomiita et al. in 2018.

Methods: A retrospective observational study was conducted at a pediatric rheumatology reference unit between 2008 and 2024. Databases were reviewed and demographic, clinical, analytical and therapeutic data were collected from patients with a suspected diagnosis of pediatric SS.

Results: Twelve patients with a diagnosis of SS were included (Table 1). Half were female and 92% were Caucasian, with a median (min-max) age at symptom onset of 8 (3-12) years. Association with other pathology in 17%: juvenile idiopathic arthritis (n=1) and systemic lupus erythematosus (n=1). The main manifestations at the beginning were musculoskeletal (67%), followed by ocular (17%), recurrent parotitis (17%), livedo reticularis (17%) and Raynaud’s phenomenon (17%). During the course of the disease, a higher percentage of patients developed the typical sicca syndrome symptoms (xerophthalmia in 58% and xerostomia in 25%). However, the predominant manifestations during follow-up were musculoskeletal (83%), with renal involvement also being important (25%). At the serological level, ANA antibody positivity was the most frequent (92%), followed by anti-Ro antibodies (75%) and to a lesser extent, rheumatoid factor (42%) and anti-La (33%). In terms of treatment, 83% of patients were treated with hydroxychloroquine (HCQ) with good response. Regarding the criteria, only 42% of the patients met the 2016 ACR/EULAR classification criteria. Diagnostic criteria of Tomiita et al. (2018) were met by 83%, classified as: definite (33%), probable (25) and possible (25%).

Conclusion: As in the literature, in our series the most frequent symptomatology at the beginning was musculoskeletal (67%). Recurrent parotitis has also been described as a distinctive manifestation in children, showing an inverse relationship between the age of onset and the incidence of parotitis. Clinical manifestations were varied, highlighting renal involvement in 3 patients. During the course of the disease, the prevalence of ocular or oral dryness increased. Most of the patients in our series were treated with HCQ (83%), 42% with systemic corticosteroid therapy and 33% with DMARDs, according to the profile of clinical manifestations. The presentation of pediatric SS is more varied, making its diagnosis more difficult and highlighting the importance of diagnostic suspicion. In this regard, we believe it is crucial to consider the classification into possible, probable, and definite diagnosis suggested by Tomiita et al., as it better fits the presentation of pediatric SS. The ACR/EULAR classification criteria are less applicable in the pediatric population due to the lower frequency of presentation as dry syndrome. All of this suggests the importance of developing new, pediatric-specific diagnostic criteria.

REFERENCES: NIL.

Acknowledgements: NIL.

Disclosure of Interests: None declared.

© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/ ). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.