Childhood-onset versus adult-onset Sjögren Syndrome: Clinical phenotype, disease activity, and predictors of organ damage in a national multicenter cohort

Kilic Konte, Elif; Sahin, Sezgin; Firat Sentürk, Esra; Yekedüz Bülbül, Aydan; Arslanoglu, Ceyda; Orhan Erkan, Mehmet; Kücükali, Batuhan; Ugur Es, Yasemin; Kara Canlioglu, Neslihan; Koker, Oya; Kilbas, Gülsah; Oguz, Gorkem; Sahin, Cansu; Selim Kul, Mehmet; Miray Kisla Ekinci, Rabia; Sümer, Gokce; Anıl Akın, Ozan; Ilgaz Tüzen, Hande; Bozkaya Yücel, Burcu; Kisaoglu, HAKAN; Emine Sonmez, Hafize; Ozen Balci, Merve; Isgüder, Rana; Sener, Seher; DEMİRKAN, Fatma; Can Eroglu, Ender; Kacmaz, Hatice; ÇAKMAK, Figen; Haslak, Fatih; Türkucar, Serkan; ÇELİKEL, Elif; Gezgin Yildirim, Deniz; DEMİR, SELCAN; SAĞ, ERDAL; BAŞARAN, HALİDE; Kasap Demir, Belde; KILIÇ GÜLTEKİN, SARA; KALYONCU, MUKADDES; Balat, Ayse; Aktay Ayaz, Nuray; Pac Kisaarslan, Aysenur; SÖZERİ, Betül; YÜKSEL, SELÇUK; UĞURLU, SERDAL; KASAPÇOPUR, ÖZGÜR

doi:10.1016/j.semarthrit.2026.153060

Childhood-onset versus adult-onset Sjögren Syndrome: Clinical phenotype, disease activity, and predictors of organ damage in a national multicenter cohort

Kilic Konte E., Sahin S., Firat Sentürk E., Yekedüz Bülbül A., Arslanoglu C., Orhan Erkan M., ...Daha Fazla

Seminars in Arthritis and Rheumatism, cilt.80, 2026 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 80
Basım Tarihi: 2026
Doi Numarası: 10.1016/j.semarthrit.2026.153060
Dergi Adı: Seminars in Arthritis and Rheumatism
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CINAHL, EMBASE, MEDLINE
Anahtar Kelimeler: Age of onset, Clinical phenotype, ESSDAI, Parotitis, Sjögren syndrome, SSDDI
Karadeniz Teknik Üniversitesi Adresli: Evet

Özet

Objectives: To compare the clinical, immunological, and diagnostic characteristics of childhood-onset (coSS) versus adult-onset Sjögren syndrome (aoSS), assess disease activity and damage accrual, and identify independent predictors of organ damage. Methods: This national, multicenter retrospective cohort included 197 patients, 115 (58.4%) with coSS and 82 (41.6%) with aoSS. Clinical, laboratory, and outcome data were compared between groups. Disease activity and damage were evaluated using the ESSDAI and SSDDI, respectively. Damage was defined as SSDDI ≥1. Logistic regression analyses were used to identify independent predictors of damage. Results: Female predominance was more pronounced in aoSS (93.9% vs 84.3%, p=0.04), while diagnostic delay was significantly longer in coSS (8.1 vs 5.4 months, p=0.001). Fulfillment of the 2016 ACR/EULAR criteria at baseline was lower in coSS (73.0% vs 98.8%, p<0.001). coSS was characterized by more symptomatic parotitis, cervical lymphadenopathy, constitutional symptoms, and Raynaud phenomenon, whereas sicca symptoms, anti-Ro and RF positivity, leukopenia, lymphopenia, and pulmonary involvement were more frequent in aoSS. Baseline ESSDAI was higher in coSS (9.3 vs 5.0, p<0.001), whereas SSDDI at last visit was lower (p=0.002). Older age at symptom onset (OR=1.044, p<0.001), higher baseline ESSDAI (OR=1.121, p=0.002), and leukopenia (OR=2.657, p=0.046) independently predicted damage, while RF positivity was protective (OR=0.343, p=0.008). Conclusion: coSS displays a distinct clinical phenotype and higher initial disease activity than aoSS, yet accrues less damage over time. These results highlight the need for pediatric-specific classification criteria and underscore the importance of baseline disease activity, leukopenia, and RF positivity as key predictors for guiding damage-oriented risk stratification across the SS spectrum.