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抗 MDA5 抗体阳性炎性肌病患者的临床特征及抗体滴度变化。

Clinical characteristics and change in the antibody titres of patients with anti-MDA5 antibody-positive inflammatory myositis.

机构信息

Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan.

出版信息

Rheumatology (Oxford). 2017 Sep 1;56(9):1492-1497. doi: 10.1093/rheumatology/kex188.

Abstract

OBJECTIVE

The aim of this study was to evaluate the clinical characteristics of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive inflammatory myositis, and the change in anti-MDA5 antibody titres before and after onset.

METHOD

For 105 PM/DM patients, newly diagnosed in our hospital within the period 2008-2016, serum anti-MDA5 antibody levels were measured at diagnosis and after treatment by ELISA using the MESACUP anti-MDA5 test. The relationships between anti-MDA5 antibody levels and clinical manifestations, laboratory data, and mortality were examined.

RESULT

Compared with patients who were anti-MDA5 antibody negative, those who were antibody positive demonstrated more frequent dermatitis, clinically amyopathic DM, interstitial lung disease and rapid-progressive interstitial lung disease, as well as significantly higher serum ferritin, significantly lower creatine kinase and aldolase, and significantly less frequent ANA (⩾1:160) and anti-cytoplasmic pattern of ANA staining positivity. Anti-MDA5 antibody titres were examined before disease onset in two patients; one showed antibody positivity with low titres 2 years earlier, while both exhibited increased titres at onset. Anti-MDA5 antibody titres declined significantly less in survivors than in non-survivors after treatment; however, there was no significant difference between the two groups when the rate was compared at 2 months after treatment.

CONCLUSION

An initial decrease in anti-MDA5 antibody titre after commencement of treatment was observed in most of the patients, including in fatal cases, suggesting that this may not necessarily be a useful marker for treatment of patients with DM.

摘要

目的

本研究旨在评估抗黑色素瘤分化相关基因 5(MDA5)抗体阳性炎性肌病患者的临床特征,以及发病前后抗 MDA5 抗体滴度的变化。

方法

对我院 2008 年至 2016 年期间新诊断的 105 例皮肌炎/多发性肌炎患者,采用 MESACUP anti-MDA5 试验酶联免疫吸附法(ELISA)在诊断时和治疗后检测血清抗 MDA5 抗体水平。检测抗 MDA5 抗体水平与临床表现、实验室数据和死亡率之间的关系。

结果

与抗 MDA5 抗体阴性患者相比,抗体阳性患者更常出现皮炎、临床无肌病性皮肌炎、间质性肺病和快速进展性间质性肺病,血清铁蛋白显著升高,肌酸激酶和醛缩酶显著降低,抗核抗体(≥1:160)和抗细胞质型抗核抗体染色阳性率显著降低。两名患者在发病前检查了抗 MDA5 抗体滴度;其中 1 例发病前 2 年抗体阳性,滴度较低,另 1 例发病时抗体滴度升高。治疗后,幸存者的抗 MDA5 抗体滴度下降幅度明显低于非幸存者;然而,在治疗后 2 个月比较时,两组之间没有显著差异。

结论

大多数患者(包括死亡病例)在开始治疗后观察到抗 MDA5 抗体滴度的初始下降,这表明这可能不是治疗 DM 患者的有用标志物。

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