Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis.
Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis.
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中国皮肌炎队列中抗 MDA5 相关快速进展性间质性肺病预后不良的预测因素
DOI：
10.1155/2020/2024869
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发表时间：
2020
影响因子：
4.1
通讯作者：
Sun X
中科院分区：
文献类型：
--
作者：
Li Y;Li Y;Wu J;Miao M;Gao X;Cai W;Shao M;Zhang X;Xu Y;Cong L;He J;Sun X
目的抗黑色素瘤分化相关蛋白5（anti-MDA 5）自身抗体在皮肌炎（DM）患者中与快速进展性间质性肺病（RP ILD）相关。我们的研究旨在确定抗MDA 5相关RP-ILD的临床特征和预后因素。方法在北京大学人民医院确诊的抗-MDA 5相关DM（aMDA 5-DM）患者。通过免疫印迹法测定抗MDA 5抗体的存在。采用Kaplan-Meier、卡方检验、单变量和多变量数据分析。结果213例DM合并临床无肌病性皮肌炎（CADM）患者中，aMDA 5-DM占20.7%（44/213）。在aMDA 5-DM患者中，63.6%（28/44）被确定为患有抗MDA 5相关RP-ILD。在随访期间，32.1%（9/28）的抗MDA 5相关RP-ILD患者死于呼吸衰竭。我们确定老年和甲周红斑是RP-ILD死亡率的两个独立危险因素。发病时年龄≥ 57岁与抗MDA 5相关RP-ILD患者的生存率差显著相关（P = 0. 02），而有甲周红斑的患者的生存率高于无甲周红斑的患者（P <0. 05）。结论抗MDA 5相关RP-ILD与DM/CADM患者的生存率显著相关。应给予抗MDA 5相关RP-ILD患者更有效的干预，尤其是老年患者和无甲周红斑的患者。
Objective Antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibody has been reported in dermatomyositis (DM) to be associated with rapidly progressive interstitial lung disease (RP-ILD). Our study is aimed at determining the clinical characteristics and prognostic factors underpinning anti-MDA5-associated RP-ILD. Methods Patients with anti-MDA5-associated DM (aMDA5-DM) were identified at the Peking University People's Hospital. The presence of anti-MDA5 antibody was determined by immunoblotting. Kaplan-Meier, chi-square test, univariate, and multivariate data analyses were used. Results Out of 213 patients with DM and clinically amyopathic dermatomyositis (CADM), 20.7% (44/213) of patients were identified as aMDA5-DM. Amongst the aMDA5-DM patients, 63.6% (28/44) were identified as having anti-MDA5-associated RP-ILD. During the follow-up, 32.1% (9/28) of patients with anti-MDA5-associated RP-ILD died of respiratory failure. We identified older age and periungual erythema as two independent risk factors for RP-ILD mortality. Age ≥ 57 years at disease onset was significantly associated with poor survival (P = 0.02) in patients with anti-MDA5-associated RP-ILD, while patients with periungual erythema had a better survival rate than those without periungual erythema (P < 0.05). Conclusions Anti-MDA5-associated RP-ILD is significantly associated with poor survival rates in DM/CADM patients. More effective intervention should be administered to anti-MDA5-associated RP-ILD patients, especially to senior patients and those without periungual erythema.
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