Elif Kilic Konte, Sezgin Sahin, Esra Firat Sentürk, Aydan Yekedüz Bülbül, Ceyda Arslanoglu, Mehmet Orhan Erkan, Batuhan Kücükali, Yasemin Ugur Es, Neslihan Kara Canlioglu, Oya Koker, Gülsah Kilbas, Gorkem Oguz, Cansu Sahin, Mehmet Selim Kul, Rabia Miray Kisla Ekinci, Gokce Sümer, Ozan Anıl Akın, Hande Ilgaz Tüzen, Burcu Bozkaya Yücel, Hakan Kisaoglu, Hafize Emine Sonmez, Merve Ozen Balci, Rana Isgüder, Seher Sener, Fatma Gül Demirkan, Ender Can Eroglu, Hatice Melisa Kacmaz, Figen Cakmak, Fatih Haslak, Serkan Türkucar, Elif Celikel, Deniz Gezgin Yildirim, Selcan Demir, Erdal Sag, Ozge Basaran, Belde Kasap Demir, Sara S Kilic, Mukaddes Kalyoncu, Ayse Balat, Nuray Aktay Ayaz, Aysenur Pac Kisaarslan, Betül Sozeri, Selcuk Yüksel, Serdal Ugurlu, Ozgur Kasapcopur
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.
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.