TOPLINE:

Patients with childhood-onset Sjögren disease exhibited distinct disease patterns characterized by high or low disease activity and high or low symptom burden during adolescence and young adulthood. A notable proportion developed glandular damage and lymphoma.

METHODOLOGY:

• Researchers conducted a combined retrospective and prospective analysis to evaluate long-term outcomes in 30 children and young individuals aged 13-36 years with childhood-onset Sjögren disease (mean age at onset, 12.7 years; 93% female) who were followed into adulthood.
• All participants were diagnosed with childhood-onset Sjögren disease based on expert opinion and were followed up for a mean of 10 years post-diagnosis.
• Disease activity, symptom severity, and damage trajectories were assessed using validated scores (European Alliance of Associations for Rheumatology [EULAR] Sjögren’s Syndrome Disease Activity Index, EULAR Sjögren’s Syndrome Patient Reported Index, and Sjögren’s Syndrome Disease Damage Index, respectively).
• The cohort was assigned clinical phenotypes using the Florida Scoring System at disease onset and stratified using the Newcastle Sjögren’s Stratification Tool at the last assessment.

TAKEAWAY:

• At disease onset, the most common manifestations were fatigue (73%), arthralgia (70%), dryness (57%), swelling of the parotid and submandibular glands (50%), and skin rashes (33%); a diagnostic delay of more than 3 years from symptom onset was associated with a higher prevalence of reported dryness than a diagnosis within 3 years (100% vs 38%; P = .0014).
• Children and young people with childhood-onset Sjögren disease demonstrated two distinct trajectories for disease activity and symptoms: high vs low scores on the disease activity index (mean, 3.9 vs 0.8; P < .0001) and high vs low scores on the symptom trajectory index (mean, 5.6 vs 3.1; P = .036), respectively.
• At the last assessment, 17 (57%) of 30 patients had accumulated damage, defined as having a score of 1 or more on the Sjögren’s Syndrome Disease Damage Index, and four (13%) developed lymphoma.
• The overall extent of damage did not differ significantly by disease activity or symptom trajectory.

IN PRACTICE:

“This finding highlights the disease heterogeneity and potential need for tailored management approaches to address disease control and impact of symptoms,” the authors wrote.

SOURCE:

The study was led by Coziana Ciurtin, PhD, Centre for Adolescent Rheumatology, Department of Ageing, Rheumatology and Regenerative Medicine, University College London, London, England. It was published online on November 19, 2025, in The Lancet Rheumatology.

LIMITATIONS:

The main limitations of this study include the small sample size, partial retrospective data collection, and single-center study design. Additionally, the analysis of symptom trajectory was limited to only 73% of individuals. 

DISCLOSURES:

This study did not receive any specific funding. Some authors received grants or were supported by the National Institute of Heath Research University College London Hospital Biomedical Research Centre, Versus Arthritis, and Action Medical Research/Life Arc. Two authors reported receiving research grants, speaker honoraria, and support for attending meetings and having other ties with various pharmaceutical companies.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.