Assessment of Mortality in Autoimmune Myositis With and Without Associated Interstitial Lung Disease
Assessment of Mortality in Autoimmune Myositis With and Without Associated Interstitial Lung Disease
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DOI：
10.1007/s00408-016-9896-x
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发表时间：
2016-10-01
期刊：
影响因子：
5
通讯作者：
Danoff, Sonye
中科院分区：
文献类型：
--
作者：
Johnson, Cheilonda;Pinal-Fernandez, Iago;Danoff, Sonye
在患有自身免疫性肌炎的患者中，相关的间质性肺病（MA-ILD）是已知的过度发病率和死亡率的贡献者。最近关于特发性炎性肌病生存率的数据主要来自亚洲和欧洲，差异很大。我们试图研究美国一个大型肌炎队列的死亡率，特别关注相关ILD的影响。对来自约翰霍普金斯肌炎中心的自身免疫性肌炎（多发性肌炎[PM]、皮肌炎[DM]或临床无肌病性皮肌炎[CADM]）参与者进行了横断面分析。评估的主要结局是全因死亡率。采用Kaplan-Meier检验估计累积死亡率;采用考克斯比例风险模型比较各组生存率的差异。831名参与者被纳入研究，中位随访时间为4.5年。438例（53%）患有PM，362例（43%）患有DM，31例（4%）患有CADM。94例（11%）受试者具有临床明显ILD。共有51名参与者死亡（6%）。在无ILD的患者中，1年、5年和10年的生存率分别为99%、95%和90%。在ILD患者中，1年、5年和10年的生存率分别为97%、91%和81%。与无ILD的受试者相比，ILD受试者的死亡风险在统计学上显著更高（HR 2.13。我们分析了已知最大的自身免疫性肌炎患者队列之一，发现与没有临床明显ILD的患者相比，具有临床明显ILD的患者的死亡率显著更高。我们的研究结果表明，ILD仍然是炎性肌病患者死亡率的一个重要来源，因此应进行筛查和积极治疗。
Among patients with autoimmune myositis, associated interstitial lung disease (MA-ILD) is a known contributor of excess morbidity and mortality. Recent data on survival in idiopathic inflammatory myopathies originate primarily in Asia and Europe and vary widely. We sought to examine mortality in a large U.S. myositis cohort focusing in particular on the impact of associated ILD.A cross-sectional analysis of participants from the Johns Hopkins Myositis Center with autoimmune myositis (polymyositis [PM], dermatomyositis [DM], or clinically amyopathic dermatomyositis [CADM]) was conducted. The primary outcome assessed was all-cause mortality. Cumulative mortality rates were estimated using the Kaplan-Meier test; the Cox proportional hazards model was used to compare group differences in survival.Eight hundred and thirty-one participants were included with a median follow-up time of 4.5 years. Four hundred thirty-eight (53 %) had PM, 362 (43 %) had DM, and 31 (4 %) had CADM. Ninety-four (11 %) participants had clinically evident ILD. Overall, 51 participants died (6 %). In those without ILD, the survival rates at 1, 5, and 10 years were 99, 95, and 90 %, respectively. In those with ILD, the survival rates at 1, 5, and 10 years were 97, 91, and 81 %, respectively. The risk of death was statistically significantly higher among participants with ILD compared to those without ILD (HR 2.13. 95 % CI 1.06-4.25; p = 0.03).We analyzed one of the largest known cohorts of patients with autoimmune myositis and found significantly higher mortality rates among those with clinically evident ILD compared to those without clinically evident ILD. Our results suggest that ILD remains an important and significant source of mortality in patients with inflammatory myopathies and as such should be screened for and treated aggressively.