Early illness features associated with mortality in the juvenile idiopathic inflammatory myopathies.
Early illness features associated with mortality in the juvenile idiopathic inflammatory myopathies.
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DOI：
10.1002/acr.22212
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发表时间：
2014-05
影响因子：
4.7
通讯作者：
Childhood Myositis Heterogeneity Collaborative Study Group
中科院分区：
文献类型：
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作者：
Huber AM;Mamyrova G;Lachenbruch PA;Lee JA;Katz JD;Targoff IN;Miller FW;Rider LG;Childhood Myositis Heterogeneity Collaborative Study Group
由于青少年特发性炎症性肌病（JIIM）是潜在威胁生命的系统性自身免疫性疾病，我们研究了JIIM死亡率的危险因素。405例患者（329例青少年皮肌炎[JDM]， 30例青少年多发性肌炎[JPM]， 46例青少年结缔组织病相关肌炎[JCTM]）的死亡率状况纳入全国方案。使用美国人口统计数据计算标准化死亡率（SMR）。Cox回归用于评估与死亡率的单变量关联，随机生存森林（RSF）分类和Cox回归用于评估多变量关联。在17例死亡（总死亡率4.2%）中，8例（2.4%）为JDM患者。死亡与肺系统有关，主要是间质性肺疾病（ILD） 7例，胃肠道3例，多系统3例，病因不明4例。JIIM的总体SMR为14.4[95%可信区间（CI） 12.2, 16.5]， JDM的SMR为8.3[95%可信区间（CI） 6.4, 10.3]。单变量分析中死亡率最高的危险因素包括临床亚组（JCTM、JPM）、抗合成酶自身抗体、诊断时年龄较大、ILD和诊断时雷诺现象。在多变量分析中，临床亚组、发病时疾病严重程度、诊断时年龄、体重减轻和诊断延迟是RSF最重要的预测因子；多变量Cox回归分析确定临床亚组和发病时疾病严重程度。JIIM患者的总体死亡率较高，一些早期疾病特征被确定为危险因素。临床亚群、抗合成酶自身抗体、诊断年龄和ILD也被认为是成人肌炎的死亡危险因素。
Because juvenile idiopathic inflammatory myopathies (JIIM) are potentially life-threatening systemic autoimmune diseases, we examined risk factors for JIIM mortality. Mortality status was available for 405 patients (329 juvenile dermatomyositis [JDM], 30 juvenile polymyositis [JPM], 46 juvenile connective tissue disease–associated myositis [JCTM]) enrolled in nationwide protocols. Standardized mortality ratios (SMR) were calculated using United States population statistics. Cox regression was used to assess univariable associations with mortality, and random survival forest (RSF) classification and Cox regression for multivariable associations. Of 17 deaths (4.2% overall mortality), 8 (2.4%) were in JDM patients. Death was related to the pulmonary system, primarily interstitial lung disease (ILD), in 7 patients, gastrointestinal in 3, multisystem in 3, and of unknown etiology in 4 patients. The SMR for JIIM overall was 14.4 [95% confidence interval (CI) 12.2, 16.5] and 8.3 [95% CI 6.4, 10.3] for JDM. The top mortality risk factors in the univariable analysis included clinical subgroup (JCTM, JPM), anti-synthetase autoantibodies, older age at diagnosis, ILD and Raynaud’s phenomenon at diagnosis. In multivariable analyses, clinical subgroup, illness severity at onset, age at diagnosis, weight loss and delay to diagnosis were the most important predictors from RSF; clinical subgroup and illness severity at onset were confirmed by multivariable Cox regression. Overall mortality was higher in JIIM patients, and several early illness features were identified as risk factors. Clinical subgroup, anti-synthetase autoantibodies, older age at diagnosis, and ILD are also recognized as mortality risk factors in adult myositis.