https://scholars.lib.ntu.edu.tw/handle/123456789/525020
標題: | Fifteen-year experience of pediatric-onset mixed connective tissue disease | 作者: | Tsai Y.-Y. YAO-HSU YANG HSIN-HUI YU LI-CHIEH WANG JYH-HONG LEE BOR-LUEN CHIANG |
公開日期: | 2010 | 卷: | 29 | 期: | 1 | 起(迄)頁: | 53-58 | 來源出版物: | Clinical Rheumatology | 摘要: | The aim of this study was to investigate the initial clinical manifestations, laboratory data, complications, and outcomes of patients with pediatric-onset mixed connective tissue disease (MCTD) in Taiwan. We reviewed medical charts of patients younger than 18 years with a diagnosis of mixed connective tissue disease based on the criteria of Kasukawa (1) at the pediatric department of National Taiwan University Hospital from 1993 to 2008. A total of 12 patients were included. All of the patients were female. The mean age at disease onset was 10.7 years (range 6.5 to 14 years). The most common symptoms at disease onset were polyarthritis (7/12 patients) and Raynaud's phenomenon (7/12 patients). The clinical symptoms changed with time, and other symptoms encompassing the criteria for MCTD developed sequentially. Inflammatory manifestations (arthritis, fever, and skin rash) improved following treatment, whereas sclerodermatous features (sclerodactyly, esophageal disease, and vasculopathy) persisted and were often unresponsive to therapy. The organ involvement-free rates at 2 years, 5 years, and 10 years were 91.7%, 78.6%, and 52.4%, respectively. In this retrospective study, sclerodermatous changes of internal organs were a poor prognostic factor in our population, and we emphasize that long-term follow-up is necessary, and appropriate treatment should be applied to improve the outcomes. ? 2009 Clinical Rheumatology. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-72949120213&doi=10.1007%2fs10067-009-1276-y&partnerID=40&md5=5c6b8830dc4a1a022fe70177b661b687 https://scholars.lib.ntu.edu.tw/handle/123456789/525020 |
ISSN: | 0770-3198 | DOI: | 10.1007/s10067-009-1276-y | SDG/關鍵字: | autoantibody; azathioprine; calcium channel blocking agent; corticosteroid; cyclophosphamide; cyclosporin; double stranded DNA antibody; hydroxychloroquine; La antibody; methotrexate; nonsteroid antiinflammatory agent; penicillamine; Ro antibody; Sc170 antibody; Sm antibody; unclassified drug; adolescent; arthritis; article; cerebellum infarction; child; clinical article; controlled study; disease duration; endoscopy; esophagus disease; esophagus hypomotility; female; fever; finger swelling; gangrene; headache; hemolytic anemia; hepatic encephalopathy; human; hyperammonemia; kidney failure; leukopenia; liver cirrhosis; lung disease; lymphocytic infiltration; mixed connective tissue disease; myositis; pediatrics; pericarditis; polyarthritis; portal hypertension; preschool child; priority journal; prognosis; psychosis; pyelonephritis; rash; Raynaud phenomenon; retrospective study; sclerodactyly; scleroderma; seizure; Sjoegren syndrome; swelling; symptomatology; systemic lupus erythematosus; telangiectasia; thrombocytopenia; treatment response; tuberculous meningitis; upper gastrointestinal bleeding; vascular disease; vasculitis; Adolescent; Age of Onset; Arthritis; Child; Disease Progression; Female; Follow-Up Studies; Humans; Mixed Connective Tissue Disease; Prognosis; Raynaud Disease; Retrospective Studies; Risk Factors; Scleroderma, Systemic; Severity of Illness Index; Taiwan |
顯示於: | 醫學系 |
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