Predicting the Course of Juvenile Dermatomyositis Significance of Early Clinical and Laboratory Features
Predicting the Course of Juvenile Dermatomyositis Significance of Early Clinical and Laboratory Features
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DOI：
10.1002/art.23960
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发表时间：
2008-11-01
影响因子：
--
通讯作者：
Feldman, Brian M.
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文献类型：
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作者：
Stringer, Elizabeth;Singh-Grewal, Davinder;Feldman, Brian M.
Objective.幼年型皮肌炎是一种罕见的儿童慢性炎症性疾病。青少年糖尿病的临床病程似乎是可变的，并且对病程的预测因子知之甚少。本研究的目的是描述青少年糖尿病的临床过程，并确定早期的临床和实验室特征是否可用于预测缓解时间和/或疾病过程。将84例青少年DM患者的临床和实验室数据前瞻性地输入数据库（1990-2005年）。缓解定义为停药6个月内无活动性皮疹、虚弱或肌酶水平升高的临床状态。病程被定义为单相、多相或慢性。在诊断时以及诊断后3个月和6个月对数据进行回顾，以确定缓解时间和/或疾病进程的预测因素。中位缓解时间为4.67年。60%的患者为慢性病程，37%为单相病程，3%为多相病程。3个月时出现皮疹（最明显的表现为Gottron丘疹）是较长缓解时间的最早预测因素（相对危险度[RR] 0.55 [95%置信区间（95% CI）0.37-0.81]，P = 0.002）。在6个月时，甲襞异常和皮疹的存在也预测了较长的缓解时间（RR 0.35 [95%CI 0.14-0.74]，P = 0.003）。我们无法确定疾病进程的预测模型。我们队列中的大多数患者有慢性疾病病程。病程早期戈特朗丘疹和甲襞异常的持续存在与较长的什一税缓解相关。
Objective. Juvenile dermatomyositis (DM) is a rare chronic inflammatory disease of childhood. The clinical course of juvenile DM appears to be variable, and little is known about predictors of the disease course. The aims of this study were to describe the clinical course of juvenile DM and to determine whether early clinical and laboratory features can be used to predict the time to remission and/or the disease course.Methods. Clinical and laboratory data from a cohort of 84 patients with juvenile DM were prospectively entered into a database (1990-2005). Remission was defined as a clinical state of no active skin rash, weakness, or elevated muscle enzyme levels for 6 months off medication. The disease course was defined as monophasic, polyphasic, or chronic. Data were reviewed at the time of diagnosis and at 3 months and 6 months after the diagnosis to determine predictors of the time to remission and/or the disease course.Results. The median time to remission was 4.67 years. Sixty percent of patients had a chronic course, 37% a monophasic course, and 3% a polyphasic course. The presence of rash (most strongly indicated by Gottron's papules) at 3 months was the earliest predictor of a longer time to remission (relative risk [RR] 0.55 [95% confidence interval (95% CI) 0.37-0.81], P = 0.002). At 6 months, the presence of nailfold abnormalities and rash also predicted a longer time to remission (RR 0.35 [95% CI 0.14-0.74], P = 0.003). We were unable to determine a prediction model of disease course.Conclusion. The majority of patients in our cohort had a chronic disease course. The persistence of Gottron's papules and nailfold abnormalities early in the disease course was associated with a longer tithe to remission.