Childhood Sjögren disease differs sharply from adult disease in Italian cohort

Childhood-onset Sjögren disease presents with a markedly different clinical picture than the adult form of the condition, according to a nationwide Italian multicentre study published in Arthritis Research & Therapy. In a cross-sectional comparison of 102 children with childhood-onset Sjögren disease and 140 adults with adult-onset disease, children showed far more frequent systemic inflammatory involvement, while the dryness symptoms that typically define the condition in adults were much less prominent in the pediatric group.

The study recruited children fulfilling the 2016 ACR/EULAR classification criteria for Sjögren disease from 22 Italian pediatric rheumatology centres, and compared them with adult-onset patients drawn from a tertiary referral centre. The authors note that childhood-onset Sjögren disease is a rare autoimmune disease whose phenotype remains incompletely characterized, with limited longitudinal data, no validated classification criteria specific to children, and limited evidence to guide treatment decisions.

The most striking difference concerned the salivary glands. Recurrent parotid enlargement was present in 44% of the children compared with 13% of the adults, a difference the authors report as statistically significant with p < 0.0001. Cutaneous involvement was also more common in children, affecting 25% versus 8% of adults (p < 0.0001), and lymphadenopathy appeared in 24% of children against 9% of adults (p = 0.001).

Musculoskeletal and constitutional features followed the same pattern. Arthritis was recorded in 17.7% of the pediatric cohort compared with 6.4% of the adult cohort (p = 0.006), and fever occurred in 12% of children versus 3% of adults (p = 0.006). These findings collectively point to a more actively inflammatory, systemic presentation in childhood-onset disease.

In contrast, the classic sicca manifestations were significantly less common among the children. Xerostomia, or dry mouth, affected 45% of the pediatric patients compared with 94% of the adults, and xerophthalmia, or dry eyes, affected 36% versus 86%, with both comparisons reaching p < 0.0001.

Serological and disease-activity measures also diverged between the two groups. Children had a higher median ESSDAI score, a measure of systemic disease activity, at 5.5 compared with 1.0 in adults (p < 0.0001), and higher serum IgG levels.

The study also followed the pediatric cohort longitudinally over a median of 26.4 months. During this period, 87.5% of the children initiated systemic treatment, underscoring how frequently the disease requires therapy beyond local measures. In exploratory analyses, a higher baseline erythrocyte sedimentation rate was independently associated with a shorter time to the initiation of systemic treatment, suggesting that this laboratory marker may help identify children likely to need earlier therapeutic intervention.

The authors conclude that compared with adult-onset Sjögren disease, childhood-onset disease shows more frequent systemic inflammatory involvement and less prominent sicca manifestations. They emphasise that pediatric-specific classification criteria, outcome measures and prospective studies remain crucial unmet needs for optimising management and improving long-term outcomes. As a cross-sectional comparison supplemented by descriptive longitudinal follow-up from a single country’s referral network, the study provides a detailed phenotype map of the childhood disease, but the authors frame their treatment-related findings as exploratory and call for prospective research to build on them.

Subject of Research: Clinical phenotype, disease course and treatment patterns of childhood-onset Sjögren disease compared with adult-onset disease in an Italian multicentre cohort

Article Title: Childhood-onset Sjögren disease : clinical phenotype compared with adult-onset disease, disease course and treatment patterns in a nationwide Italian multicentre pediatric cohort

Article References: Riccio, F., Ancona, S., Campus, S., Marino, A., Simonini, G., Baldo, F., Marrani, E., Ibba, V., Mauro, A., Meneghel, A., Rigante, D., Del Giudice, E., Pastore, S., Manfrè, V., Civino, A., Covizzi, C., Naddei, R., Uva, A., Loconte, R., … Malattia, C. (2026). Childhood-onset Sjögren disease : clinical phenotype compared with adult-onset disease, disease course and treatment patterns in a nationwide Italian multicentre pediatric cohort. Arthritis Research & Therapy. https://doi.org/10.1186/s13075-026-03925-4

Image Credits: AI Generated

DOI: 10.1186/s13075-026-03925-4

Keywords: Sjögren disease, pediatric rheumatology, childhood-onset disease, autoimmune disease, sicca symptoms, ESSDAI, parotid enlargement, anti-SSA antibodies, systemic treatment, Italian cohort, cross-sectional study, disease activity