Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis.
Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis.
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DOI：
10.1002/acr.22728
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发表时间：
2016-05
影响因子：
4.7
通讯作者：
Aggarwal R
中科院分区：
文献类型：
--
作者：
Moghadam-Kia S;Oddis CV;Sato S;Kuwana M;Aggarwal R
临床无肌病性皮肌炎（CADM）是皮肌炎（DM）的一个亚类，表现为DM的特征性皮疹，无客观肌无力。亚洲研究报告称，CADM患者的抗黑色素瘤分化相关基因5（抗MDA-5）自身抗体与间质性肺病（ILD）相关，尤其是快速进展性ILD（RPILD）。这些关联在美国肌炎患者中尚未建立。本研究的目的是确定抗MDA-5自身抗体与美国CADM和经典DM患者ILD、RPILD和生存率的相关性。CADM患者在匹兹堡大学肌炎中心数据库中确定，并与经典DM对照组1：1（性别和年龄）匹配。血清酶联免疫吸附法测定抗MDA-5。采用Kaplan-Meier、对数秩和卡方检验进行分析。我们确定了61例CADM患者（62%为女性，平均年龄48.2岁）和61例经典DM对照（64%为女性，平均年龄44.8岁）。抗MDA-5阳性、ILD和RPILD的频率在2个队列中相似（MDA-5阳性：CADM 13.1% [8/61]和DM 13.1% [8/61]，ILD阳性：CADM 31.1% [19/61]和DM 26.2% [16/61]，RPILD阳性：CADM 8.2% [61例中的5例]和DM 5% [61例中的3例]; P=1、0.55和0.46）。抗MDA-5阳性与ILD显著相关，因为50%的MDA-5阳性受试者（16例中的8例）患有ILD，而MDA-5阴性受试者（106例中的27例; P=0.04）患有ILD。抗MDA-5抗体与RPILD密切相关（P < 0.001）。与抗MDA-5阴性患者相比，抗MDA-5阳性ILD患者的基线肺功能检测变量更差。抗MDA-5阳性与生存率差显著相关（P=0.007）。抗MDA-5抗体与美国典型DM和CADM患者的ILD、RPILD、肺结局恶化和生存率显著相关。
Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) presenting with the characteristic rash of DM without objective muscle weakness. Asian studies report that anti–melanoma differentiation–associated gene 5 (anti–MDA-5) autoantibody in CADM is associated with interstitial lung disease (ILD), particularly rapidly progressive ILD (RPILD). These associations have not been established in US myositis patients. The goal of our study was to determine the association of anti–MDA-5 autoantibody with ILD, RPILD, and survival in US patients with CADM and classic DM. CADM patients were identified in the University of Pittsburgh Myositis Center Database and matched 1:1 (sex and age) to classic DM controls. Anti–MDA-5 was measured by serum enzyme-linked immunosorbent assay. Kaplan-Meier, log rank, and chi-square tests were used for analysis. We identified 61 CADM patients (62% women, mean age 48.2 years) and 61 classic DM controls (64% women, mean age 44.8 years). The frequencies of anti–MDA-5-positivity, ILD, and RPILD were similar in the 2 cohorts (MDA-5 positive: CADM 13.1% [8 of 61] and DM 13.1% [8 of 61], ILD positive: CADM 31.1% [19 of 61] and DM 26.2% [16 of 61], and RPILD positive: CADM 8.2% [5 of 61] and DM 5% [3 of 61]; P=1, 0.55, and 0.46, respectively). Anti–MDA-5-positivity was significantly associated with ILD, since 50% of MDA-5–positive subjects (8 of 16) had ILD versus 25.5% of MDA-5–negative subjects (27 of 106; P=0.04). Anti–MDA-5 was strongly associated with RPILD (P < 0.001). Anti–MDA-5–positive patients with ILD had worse baseline pulmonary function testing variables compared to anti–MDA-5–negative patients. Anti–MDA-5-positivity was significantly associated with poor survival (P=0.007). Anti–MDA-5 antibody is significantly associated with ILD, RPILD, worse pulmonary outcome, and survival in US classic DM and CADM patients.