https://scholars.lib.ntu.edu.tw/handle/123456789/524995
標題: | Juvenile dermatomyositis: A 20-year retrospective analysis of treatment and clinical outcomes | 作者: | Sun C. JYH-HONG LEE YAO-HSU YANG HSIN-HUI YU LI-CHIEH WANG YU-TSAN LIN BOR-LUEN CHIANG |
公開日期: | 2015 | 出版社: | Elsevier (Singapore) Pte Ltd | 卷: | 56 | 期: | 1 | 起(迄)頁: | 31-39 | 來源出版物: | Pediatrics and Neonatology | 摘要: | Background Juvenile dermatomyositis is a rare childhood multisystem autoimmune disease involving primarily the skin and muscles, and it may lead to long-term disability. This study aimed to describe the clinical course of juvenile dermatomyositis and determine if any early clinical or laboratory features could predict outcome. Methods Medical charts of patients aged ?18 years and diagnosed with juvenile dermatomyositis (according to the criteria of Bohan and Peter) at the Pediatric Department, National Taiwan University Hospital, between 1989 and 2009 were reviewed. The endpoints for disease assessment were complete clinical response and complete clinical remission. Cox's proportional hazards model was fitted to identify important predictors of complete clinical remission. Results A total of 39 patients with juvenile dermatomyositis were reviewed. Two-thirds were females, and the mean age at disease onset was 81.97 ± 46.63 months. The most common initial presentations were Gottron's papule (82.1%) and muscle weakness (82.1%). After excluding one patient with an incomplete record, the remaining 31 patients who had muscle weakness were analyzed; among them, 22 (70.97%) achieved complete clinical response, but only six (19.4%) achieved complete clinical remission. Multivariate analysis showed that female sex, negative Gowers' sign at disease onset, and positive photosensitivity at disease onset were favorable factors to achieve complete clinical remission. Moreover, covariate-adjusted survival curves were drawn for making predictions of complete clinical remission. Only 13 (33.33%) patients were symptom free at the end of follow up, whereas the other 26 suffered from different kinds of complications. None of them developed malignancy, but two (5.13%) patients died during the follow-up period. Conclusion Factors such as male sex and Gowers' sign were unlikely to favor the achievement of complete clinical remission in juvenile dermatomyositis. Certain complications cannot be avoided, and thus more effective treatments and monitoring strategies are needed for better control of juvenile dermatomyositis. Copyright ? 2014, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-84921632120&doi=10.1016%2fj.pedneo.2014.02.006&partnerID=40&md5=521145ede762410b623026032ac44dac https://scholars.lib.ntu.edu.tw/handle/123456789/524995 |
ISSN: | 1875-9572 | DOI: | 10.1016/j.pedneo.2014.02.006 | SDG/關鍵字: | azathioprine; corticosteroid; cyclophosphamide; cyclosporin A; etanercept; hydroxychloroquine; immunoglobulin G; methotrexate; methylprednisolone; muscle enzyme; nonsteroid antiinflammatory agent; prednisolone; salazosulfapyridine; Article; calcinosis; child; clinical article; clinical feature; controlled study; corticosteroid therapy; disease assessment; disease course; drug pulse therapy; enzyme blood level; female; follow up; human; juvenile dermatomyositis; male; muscle weakness; onset age; outcome assessment; papule; photosensitivity; preschool child; prognosis; rash; remission; retrospective study; treatment response; adolescent; complication; dermatomyositis; infant; multivariate analysis; Photosensitivity Disorders; sex difference; Taiwan; Adolescent; Child; Child, Preschool; Dermatomyositis; Female; Humans; Infant; Male; Multivariate Analysis; Photosensitivity Disorders; Remission Induction; Retrospective Studies; Sex Factors; Taiwan |
顯示於: | 醫學系 |
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