Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis of 158 children.

Autor:	Ramos-Casals M; Department of Autoimmune Diseases, ICMiD, Hospital Clínic.; Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX.; Department of Medicine, University of Barcelona., Acar-Denizli N; Department of Statistics and Operations Research, Universitat Politècnica de Catalunya, Barcelona, Spain., Vissink A; Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands., Brito-Zerón P; Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX.; Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA - Sanitas, Barcelona, Spain., Li X; Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China., Carubbi F; Clinical Unit of Rheumatology, University of l'Aquila, School of Medicine, L'Aquila., Priori R; Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Rome, Italy., Toplak N; University Children's Hospital Ljubljana, University Medical Center Ljubljana, Medical Faculty of Ljubljana, Slovenia., Baldini C; Rheumatology Unit, University of Pisa, Pisa, Italy., Faugier-Fuentes E; Hospital Infantil de México Federico Gómez, Ciudad de México, México., Kruize AA; Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands., Mandl T; Department of Rheumatology, Skane University Hospital Malmö, Lund University, Lund, Sweden., Tomiita M; Department of Pediatrics, National Hospital Organization, Shimoshizu National Hospital, Yotsukaido, Japan., Gandolfo S; Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital 'Santa Maria della Misericordia', Udine, Italy., Hashimoto K; Department of Pediatrics (Pediatric Allergy and Rheumatology), Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan., Hernandez-Molina G; Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, Mexico., Hofauer B; Otorhinolaryngology, Head and Neck Surgery, Technical University Munich, Munich, Germany., Mendieta-Zerón S; Hospital Materno Infantil ISSEMyM, Toluca, México., Rasmussen A; Genes and Human Disease Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA., Sandhya P; Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India., Sene D; Service de Médecine Interne 2, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, Paris, France., Trevisani VFM; Federal University of São Paulo, Sao Paulo, Brazil., Isenberg D; Centre for Rheumatology, Division of Medicine, University College London, London, UK., Sundberg E; Pediatric Rheumatology, Astrid Lindgreńs Children Hospital, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden., Pasoto SG; Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil., Sebastian A; Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland., Suzuki Y; Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan., Retamozo S; Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX.; Instituto Modelo de Cardiología Privado SRL.; Instituto Universitario de Ciencias Biomédicas de Córdoba (IUCBC), Córdoba, Argentina., Xu B; Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China., Giacomelli R; Clinical Unit of Rheumatology, University of l'Aquila, School of Medicine, L'Aquila., Gattamelata A; Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Rome, Italy., Bizjak M; University Children's Hospital Ljubljana, University Medical Center Ljubljana, Medical Faculty of Ljubljana, Slovenia., Bombardieri S; Rheumatology Unit, University of Pisa, Pisa, Italy., Loor-Chavez RE; Hospital Infantil de México Federico Gómez, Ciudad de México, México., Hinrichs A; Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands., Olsson P; Department of Rheumatology, Skane University Hospital Malmö, Lund University, Lund, Sweden., Bootsma H; Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands., Lieberman SM; Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
Jazyk:	angličtina
Zdroj:	Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2021 Oct 02; Vol. 60 (10), pp. 4558-4567.
DOI:	10.1093/rheumatology/keab032
Abstrakt:	Objectives: To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results: Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. Conclusions: Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease. (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
Databáze:	MEDLINE
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