抗黑色素瘤分化相关基因5与美国无肌病性和肌病性皮肌炎患者的快速进展性肺病及不良生存相关。
Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis.
作者信息
Moghadam-Kia Siamak, Oddis Chester V, Sato Shinji, Kuwana Masataka, Aggarwal Rohit
机构信息
University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
Tokai University, School of Medicine, Isehara, Japan.
出版信息
Arthritis Care Res (Hoboken). 2016 May;68(5):689-94. doi: 10.1002/acr.22728.
OBJECTIVE
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.
METHODS
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.
RESULTS
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).
CONCLUSION
Anti-MDA-5 antibody is significantly associated with ILD, RPILD, worse pulmonary outcome, and survival in US classic DM and CADM patients.
目的
临床无肌病性皮肌炎（CADM）是皮肌炎（DM）的一个亚型，其特征为有DM典型皮疹但无客观的肌无力表现。亚洲的研究报告称，CADM中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疾病（ILD）相关，尤其是快速进展性ILD（RPILD）。在美国的肌炎患者中尚未证实这些关联。我们研究的目的是确定在美国CADM和典型DM患者中抗MDA - 5自身抗体与ILD、RPILD及生存率之间的关联。
方法
在匹兹堡大学肌炎中心数据库中识别出CADM患者，并按1:1（性别和年龄）与典型DM对照进行匹配。通过血清酶联免疫吸附测定法检测抗MDA - 5。采用Kaplan - Meier法、对数秩检验和卡方检验进行分析。
结果
我们识别出61例CADM患者（62%为女性，平均年龄48.2岁）和61例典型DM对照（64%为女性，平均年龄44.8岁）。两个队列中抗MDA - 5阳性、ILD和RPILD的发生率相似（抗MDA - 5阳性：CADM为13.1%[61例中的8例]，DM为13.1%[61例中的8例]；ILD阳性：CADM为31.1%[61例中的19例]，DM为26.2%[61例中的16例]；RPILD阳性：CADM为8.2%[61例中的5例]，DM为5%[61例中的3例]；P分别为1、0.55和0.46）。抗MDA - 5阳性与ILD显著相关，因为50%的抗MDA - 5阳性受试者（16例中的8例）患有ILD，而抗MDA - 5阴性受试者中这一比例为25.5%（106例中的27例；P = 0.04）。抗MDA - 5与RPILD密切相关（P < 0.001）。与抗MDA - 5阴性患者相比，抗MDA - 5阳性且患有ILD的患者基线肺功能测试变量更差。抗MDA - 5阳性与生存率低显著相关（P = 0.007）。
结论
在美国典型DM和CADM患者中，抗MDA - 5抗体与ILD、RPILD、更差的肺部结局及生存率显著相关。
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