期刊
RHEUMATOLOGY
卷 60, 期 10, 页码 4558-4567出版社
OXFORD UNIV PRESS
DOI: 10.1093/rheumatology/keab032
关键词
Sjogren's syndrome; epidemiology; autoimmune diseases; paediatrics; childhood
类别
资金
- Fondo de Investigaciones Sanitarias [INT15/00085]
- Hospital Clinic Barcelona (Ajut per a la Recerca Josep Font)
Childhood-onset primary Sjogren's syndrome is characterized by sicca features, parotid enlargement, and systemic disease, with age at diagnosis influencing the phenotypic expression of the disease. Symptom presentation includes dry mouth, dry eyes, positive ANA, anti-Ro/La antibodies, and positive RF, among others.
Objectives To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods The Big Data Sjogren 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 Sjogren 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 Sjogren'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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据