Childhood-onset versus adult-onset Sjögren Syndrome: Clinical phenotype, disease activity, and predictors of organ damage in a national multicenter cohort.
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История · было → стало
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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, Demirkan Fatma Gül, Can Eroglu Ender, Kacmaz Hatice Melisa, Cakmak Figen, Haslak Fatih, Türkucar Serkan, Celikel Elif, Gezgin Yildirim Deniz, Demir Selcan, Sag Erdal, Basaran Ozge, Kasap Demir Belde, Kilic Sara S, Kalyoncu Mukaddes, Balat Ayse, Aktay Ayaz Nuray, Pac Kisaarslan Aysenur, Sozeri Betül, Yüksel Selcuk, Ugurlu Serdal, Kasapcopur Ozgur
Аннотация
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.