Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children
| Autor: | Ramos-Casals, M., Acar-Denizli, N., Vissink, A., Brito-Zeron, P., Li, X., Carubbi, F., Priori, R., Toplak, N., Baldini, C., Faugier-Fuentes, E., Kruize, A. A., Mandl, T., Tomiita, M., Gandolfo, S., Hashimoto, K., Hernandez-Molina, G., Hofauer, B., Mendieta-Zeron, S., Rasmussen, A., Sandhya, P., Sene, D., Trevisani, V. F. M., Isenberg, D., Sundberg, E., Pasoto, S. G., Sebastian, A., Suzuki, Y., Retamozo, S., Xu, B., Giacomelli, R., Gattamelata, A., Bizjak, M., Bombardieri, S., Loor-Chavez, R. -E., Hinrichs, A., Olsson, P., Bootsma, H., Lieberman, S. M., Kostov, B., Horvath, I. -F., Szanto, A., Seror, R., Mariette, X., Kvarnstrom, M., Wahren-Herlenius, M., Praprotnik, S., Solans, R., Nordmark, G., Hammenfors, D., Brun, J. G., Gheita, T. A., Atzeni, F., Armagan, B., Kilic, L., Kalyoncu, U., Nakamura, T., Takagi, Y., Consani, S., Solorzano, F. O. |
|---|---|
| Přispěvatelé: | Translational Immunology Groningen (TRIGR), Personalized Healthcare Technology (PHT), Universitat Politècnica de Catalunya. Departament d'Estadística i Investigació Operativa, Universitat Politècnica de Catalunya. ADBD - Anàlisi de Dades Complexes per a les Decisions Empresarials |
| Rok vydání: | 2021 |
| Předmět: |
Male
Systemic disease Anti-nuclear antibody Epidemiology Autoimmune diseases Matemàtiques i estadística::Matemàtica aplicada a les ciències [Àrees temàtiques de la UPC] Disease Severity of Illness Index Parotid Gland Medicine CLASSIFICATION CRITERIA Pharmacology (medical) Registries Age of Onset biology 92 Biology and other natural sciences::92B Mathematical biology in general [Classificació AMS] Dry eyes Phenotype Sjogren's syndrome Female epidemiology Antibody medicine.symptom PAROTITIS medicine.medical_specialty Biomatemàtica Adolescent 62 Statistics::62D05 Sampling theory sample surveys [Classificació AMS] Childhood Paediatrics Humans Sjogren's Syndrome paediatrics AGE Rheumatology Peripheral nerve Rheumatoid factor autoimmune diseases Sampling (Statistics) Primary Sjögren Syndrome childhood Biomathematics Matemàtiques i estadística::Estadística aplicada::Estadística biosanitària [Àrees temàtiques de la UPC] business.industry CLINICAL-FEATURES medicine.disease Dry mouth Dermatology stomatognathic diseases biology.protein Sjogren’s syndrome CONSENSUS business Mostreig (Estadística) Parotitis |
| Zdroj: | Rheumatology, 60(10), 4558-4567. Oxford University Press |
| ISSN: | 1462-0332 1462-0324 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|