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Idiopathic inflammatory myopathies.特发性炎性肌病。
Neurol Clin. 2014 Aug;32(3):595-628, vii. doi: 10.1016/j.ncl.2014.04.007.
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Retrospective analysis of the outcome of patients with idiopathic inflammatory myopathy: a long-term follow-up study.特发性炎症性肌病患者的预后回顾性分析:一项长期随访研究。
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Early illness features associated with mortality in the juvenile idiopathic inflammatory myopathies.幼年特发性炎性肌病相关的早期疾病特征与死亡率相关。
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多发性肌炎和皮肌炎患者的住院死亡率及相关危险因素:一项回顾性病例对照研究。

Hospitalization mortality and associated risk factors in patients with polymyositis and dermatomyositis: A retrospective case-control study.

作者信息

Wu Chanyuan, Wang Qian, He Linrong, Yang Enhao, Zeng Xiaofeng

机构信息

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.

Department of Rheumatology, China-Japan Friendship Hospital, Chaoyang District, Beijing, China.

出版信息

PLoS One. 2018 Feb 23;13(2):e0192491. doi: 10.1371/journal.pone.0192491. eCollection 2018.

DOI:10.1371/journal.pone.0192491
PMID:29474373
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5824989/
Abstract

BACKGROUND

Polymyositis and dermatomyositis (PM/DM) are systemic autoimmune diseases with multiple organ involvements that manifest as muscular and cutaneous disorders, interstitial lung disease (ILD) and malignancies. However, information concerning the outcomes and associated factors for PM/DM patients who are hospitalized is limited.

METHODS

We retrospectively reviewed the medical charts of PM/DM patients admitted to a Chinese tertiary referral hospital (Peking Union Medical College Hospital, PUMCH) from 2008 to 2014. The deceased group included 63 patients who had "deceased discharge" status or were confirmed to have died within two weeks of hospital discharge. The demographic data, clinical manifestations, and direct causes of death were analyzed retrospectively. Medical records for 126 age- and sex-matched PM/DM patients were selected as controls from 982 inpatients successively admitted to the same center during the same period. In addition to the comparison of clinical manifestations between the two groups, binary logistic regression was conducted to explore the risk factors related to PM/DM mortality.

RESULTS

Over the past 6 years at PUMCH, the in-hospital mortality rate of PM/DM patients was 4.58%. The male gender and the elder patients had a high risk of death (P = 0.031 and P = 0.001 respectively). The three most frequent causes of death for PM/DM patients were pulmonary infection (35%), ILD exacerbation (21%) or both conditions (25%). Pulmonary infection (P<0.001, OR = 5.63, 95% CI, 2.37-13.36), pneumomediastinum (P = 0.041, OR = 11.02, 95%CI, 1.10-110.54), Gottron's papules (P = 0.010, OR = 3.24, 95%CI, 1.32-7.97), and elevated erythrocyte sedimentation rate (ESR) (P = 0.005, OR = 9.9, 95%CI 2.0-49.0) were independent risk factors for in-hospital mortality of PM/DM patients.

CONCLUSION

PM/DM patients continue to display high in-hospital mortality. Pulmonary infection is the strongest predictor of poor prognosis in PM/DM patients, followed by pneumomediastinum, Gottron's papules, and elevated ESR.

摘要

背景

多发性肌炎和皮肌炎(PM/DM)是累及多个器官的系统性自身免疫性疾病,表现为肌肉和皮肤病变、间质性肺病(ILD)和恶性肿瘤。然而,关于住院的PM/DM患者的预后及相关因素的信息有限。

方法

我们回顾性分析了2008年至2014年在中国一家三级转诊医院(北京协和医院,PUMCH)住院的PM/DM患者的病历。死亡组包括63例有“死亡出院”状态或在出院后两周内被证实死亡的患者。对其人口统计学数据、临床表现和直接死因进行回顾性分析。从同期在同一中心相继住院的982例患者中选取126例年龄和性别匹配的PM/DM患者的病历作为对照。除了比较两组的临床表现外,还进行二元逻辑回归以探讨与PM/DM死亡率相关的危险因素。

结果

在过去6年中,PUMCH的PM/DM患者住院死亡率为4.58%。男性和老年患者死亡风险较高(分别为P = 0.031和P = 0.001)。PM/DM患者最常见的三个死因是肺部感染(35%)、ILD加重(21%)或两者皆有(25%)。肺部感染(P<0.001,OR = 5.63,95%CI,2.37 - 13.36)、纵隔气肿(P = 0.041,OR = 11.02,95%CI,1.10 - 110.54)、Gottron丘疹(P = 0.010,OR = 3.24,95%CI,1.32 - 7.97)和红细胞沉降率(ESR)升高(P = 0.005,OR = 9.9,95%CI 2.0 - 49.0)是PM/DM患者住院死亡的独立危险因素。

结论

PM/DM患者住院死亡率仍然较高。肺部感染是PM/DM患者预后不良的最强预测因素,其次是纵隔气肿、Gottron丘疹和ESR升高。