Results of a Nationwide Multicenter Study in Childhood Sjögren Disease.
| Title: | Results of a Nationwide Multicenter Study in Childhood Sjögren Disease. |
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| Authors: | Kılbaş G; G. Kılbaş, MD, S. Ayduran, MD, S.N. Tığrak, MD, S. Türkuçar, MD, Department of Pediatric Rheumatology, Pamukkale University, Denizli.; Ayduran S; G. Kılbaş, MD, S. Ayduran, MD, S.N. Tığrak, MD, S. Türkuçar, MD, Department of Pediatric Rheumatology, Pamukkale University, Denizli.; Şener S; S. Şener, MD, E.D. Batu, MD, S. Özen, MD, Department of Pediatric Rheumatology, Hacettepe University, Ankara.; Coşkuner T; T. Coşkuner, MD, K. Ulu, MD, B. Sözeri, MD, Department of Pediatric Rheumatology, Ümraniye Training and Research Hospital, İstanbul.; Ulu K; T. Coşkuner, MD, K. Ulu, MD, B. Sözeri, MD, Department of Pediatric Rheumatology, Ümraniye Training and Research Hospital, İstanbul.; Kısaoğlu H; H. Kısaoğlu, MD, M. Kalyoncu, MD, Department of Pediatric Rheumatology, Karadeniz Technical University, Trabzon.; Aslan E; E. Aslan, MD, E. Kılıç Könte, MD, S. Şahin, MD, Ö. Kasapçopur, MD, Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul.; Könte EK; E. Aslan, MD, E. Kılıç Könte, MD, S. Şahin, MD, Ö. Kasapçopur, MD, Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul.; Arslanaoğlu C; C. Arslanaoğlu, MD, A. Paç Kısaarslan, MD, Department of Pediatric Rheumatology, Erciyes University, Kayseri.; Aydın T; T. Aydın, MD, E. Ünsal, MD, Department of Pediatric Rheumatology, Dokuz Eylül University, İzmir.; Oğuzkaya YŞ; Y. Şadırvan Oğuzkaya, MD, S.S. Kilic, MD, Department of Pediatric Rheumatology, Uludag University, Bursa.; Çakmak F; F. Çakmak, MD, N. Aktay Ayaz, MD, Department of Pediatric Rheumatology, İstanbul University, Faculty of Medicine, İstanbul.; Yıldırım DG; D. Gezgin Yıldırım, MD, S.A. Bakkaloğlu, MD, Department of Pediatric Rheumatology, Gazi University, Ankara.; Kaplan MM; M.M. Kaplan, MD, B. Çelikel Acar, MD, Department of Pediatric Rheumatology, Ankara Bilkent City Hospital, Ankara.; Tığrak SN; G. Kılbaş, MD, S. Ayduran, MD, S.N. Tığrak, MD, S. Türkuçar, MD, Department of Pediatric Rheumatology, Pamukkale University, Denizli.; Türkuçar S; G. Kılbaş, MD, S. Ayduran, MD, S.N. Tığrak, MD, S. Türkuçar, MD, Department of Pediatric Rheumatology, Pamukkale University, Denizli.; Sönmez HE; H.E. Sönmez, MD, Department of Pediatric Rheumatology, Kocaeli University, Kocaeli.; Ekinci MK; M. Kışla Ekinci, MD, Department of Pediatric Rheumatology, Çukurova University, Adana.; Öztürk K; K. Öztürk, MD, Department of Pediatric Rheumatology, İstanbul Medeniyet University, Göztepe Prof. Dr. Süleyman Yalçın City Hospital, İstanbul.; Demir F; F. Demir, MD, Department of Pediatric Rheumatology, Acıbadem Hospital, İstanbul.; Bağlan E; E. Bağlan, MD, Department of Pediatric Rheumatology, Ankara Etlik City Hospital, Ankara.; Bozkaya B; B. Bozkaya, MD, Department of Pediatric Rheumatology, Samsun Training and Research Hospital, Samsun.; Taşkın SN; S.N. Taşkın, MD, Department of Pediatric Rheumatology, Diyarbakır Children's Hospital, Diyarbakır.; Demir S; S. Demir, MD, Department of Pediatric Rheumatology, Eskişehir Osmangazi University, Eskişehir.; Sağ E; E. Sağ, MD, Department of Pediatric Rheumatology, Ankara Training and Research Hospital, Ankara.; Batu ED; S. Şener, MD, E.D. Batu, MD, S. Özen, MD, Department of Pediatric Rheumatology, Hacettepe University, Ankara.; Şahin S; E. Aslan, MD, E. Kılıç Könte, MD, S. Şahin, MD, Ö. Kasapçopur, MD, Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul.; Bakkaloğlu SA; D. Gezgin Yıldırım, MD, S.A. Bakkaloğlu, MD, Department of Pediatric Rheumatology, Gazi University, Ankara.; Kilic SS; Y. Şadırvan Oğuzkaya, MD, S.S. Kilic, MD, Department of Pediatric Rheumatology, Uludag University, Bursa.; Kısaarslan AP; C. Arslanaoğlu, MD, A. Paç Kısaarslan, MD, Department of Pediatric Rheumatology, Erciyes University, Kayseri.; Acar BÇ; M.M. Kaplan, MD, B. Çelikel Acar, MD, Department of Pediatric Rheumatology, Ankara Bilkent City Hospital, Ankara.; Kalyoncu M; H. Kısaoğlu, MD, M. Kalyoncu, MD, Department of Pediatric Rheumatology, Karadeniz Technical University, Trabzon.; Ayaz NA; F. Çakmak, MD, N. Aktay Ayaz, MD, Department of Pediatric Rheumatology, İstanbul University, Faculty of Medicine, İstanbul.; Sözeri B; T. Coşkuner, MD, K. Ulu, MD, B. Sözeri, MD, Department of Pediatric Rheumatology, Ümraniye Training and Research Hospital, İstanbul.; Ünsal E; T. Aydın, MD, E. Ünsal, MD, Department of Pediatric Rheumatology, Dokuz Eylül University, İzmir.; Kasapçopur Ö; E. Aslan, MD, E. Kılıç Könte, MD, S. Şahin, MD, Ö. Kasapçopur, MD, Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul.; Özen S; S. Şener, MD, E.D. Batu, MD, S. Özen, MD, Department of Pediatric Rheumatology, Hacettepe University, Ankara.; Yüksel S; S. Yüksel, MD, Department of Pediatric Rheumatology, Çanakkale Onsekiz Mart University, Çanakkale, Türkiye. selcukyuksel@yahoo.com. |
| Source: | The Journal of rheumatology [J Rheumatol] 2025 Nov 01; Vol. 52 (11), pp. 1141-1150. Date of Electronic Publication: 2025 Nov 01. |
| Publication Type: | Journal Article; Multicenter Study |
| Language: | English |
| Journal Info: | Publisher: Journal Of Rheumatology Publishing Co Country of Publication: Canada NLM ID: 7501984 Publication Model: Electronic Cited Medium: Internet ISSN: 1499-2752 (Electronic) Linking ISSN: 0315162X NLM ISO Abbreviation: J Rheumatol Subsets: MEDLINE |
| Imprint Name(s): | Publication: Toronto : Journal Of Rheumatology Publishing Co; Original Publication: Toronto. |
| MeSH Terms: | Sjogren's Syndrome*/diagnosis ; Sjogren's Syndrome*/drug therapy ; Sjogren's Syndrome*/epidemiology ; Sjogren's Syndrome*/complications; Antirheumatic Agents/therapeutic use ; Xerostomia/etiology ; Humans ; Female ; Male ; Child ; Retrospective Studies ; Adolescent ; Child, Preschool ; Prognosis |
| Abstract: | Objective: This nationwide, multicenter study was conducted to assess the demographic and clinical features, treatment regimens, and prognosis of primary Sjögren disease (SjD) in childhood.; Methods: This retrospective study included a total of 81 patients < 18 years of age from 21 pediatric rheumatology centers. Among these, 51 patients fulfilled the diagnosis of childhood SjD according to the 2016 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria. The remaining 30 patients-who did not fully meet these criteria but exhibited clinical and laboratory findings suggestive of SjD-were categorized as at risk for childhood SjD to highlight diagnostic challenges and the spectrum of early presentations, based on comprehensive clinical evaluation by experienced pediatric rheumatologists.; Results: The cohort consisted of 81 patients (85.2% female, 14.8% male) with a median age at symptom onset of 11.4 years and a median follow-up of 24 months. Common clinical manifestations included dry mouth, dry eyes, arthralgia, fatigue, and parotitis. Fifty-one of the 81 patients met the 2016 ACR/EULAR classification criteria, whereas the remaining 30 were classified as an at-risk group. The most common clinical findings in the at-risk group were xerostomia (90%), arthralgia (56.7%), fatigue (50%), and dry eyes (43.3%). Dry mouth and peripheral nervous system involvements were found to be higher in patients categorized as at risk (P = 0.03 and P = 0.02, respectively).; Conclusion: The current classification criteria for childhood SjD appear to be inadequate, highlighting the need for pediatric-specific criteria that more accurately reflect the distinct clinical patterns observed in children.; (Copyright © 2025 by the Journal of Rheumatology.) |
| Contributed Indexing: | Keywords: Sjögren disease; autoimmune diseases; childhood; parotitis |
| Substance Nomenclature: | 0 (Antirheumatic Agents) |
| Entry Date(s): | Date Created: 20250715 Date Completed: 20251101 Latest Revision: 20251209 |
| Update Code: | 20260130 |
| DOI: | 10.3899/jrheum.2024-1048 |
| PMID: | 40664482 |
| Database: | MEDLINE |
Journal Article; Multicenter Study