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MDA5阳性与间质性肺炎、皮肤溃疡相关

作者:MOGHADAM-KIA,et al. 翻译:北医三院梁启刚 来源:中国风湿病公众论坛 日期:2017-05-17
导读

         MDA5阳性与(快速进展)间质性肺炎、皮肤溃疡、指端溃疡和预后差相关

关键字:  MDA5 

目的

        :了解与抗黑色素瘤分化相关基因5抗体(抗MDA5)相关临床无肌病性皮肌炎(CADM)和经典DM患者的临床特点。

方法:

        从匹兹堡肌炎数据库中选出1985年至2013年CADM的患者。CADM定义为存在典型皮肌炎皮疹,没有客观的肌无力,肌酶、肌电图或肌肉活检正常或轻度异常。DM根据Bohan和Peter标准定义,与CADM患者1:1匹配(性别和年龄±5岁),并使用ELISA测定抗MDA5自身抗体水平。使用卡方和/或Mann-Whitney U检验,对CADM和DM组以及MDA5阳性和MDA5阴性组之间进行比较。

结果:

        61例CADM患者与61例DM对照组进行匹配(女性62%vs 64%;平均年龄44.8岁vs 48.2,p <0.5)。 抗MDA5出现频率在两组(13.1%)中相同,抗MDA5明显与皮肤溃疡,指端溃疡,手指浮肿和间质性肺病(ILD)相关。 大多数ILD患者出现快速进展性ILD(RPILD)导致早期死亡。相比之下CADM患者更容易出现吞咽困难,但与经典DM相比CADM没有其他相关的临床差异。

结论:

        美国CADM和DM患者的抗MDA5阳性频率相似,MDA5阳性与ILD,RPILD,皮肤溃疡,指端溃疡和预后差相关。

        END

        附原文:

OBJECTIVE

        : Todeterminethe clinical features associated with the antimelanoma differentiation-associated gene 5 antibody (anti-MDA5) in US patients with clinically amyopathic dermatomyositis (CADM) and classic DM.: Patients with CADM were consecutively selected from the University of Pittsburgh Myositis Database from 1985 to 2013. CADM was defined by a typical DM rash without objective muscle weakness and no or minimal abnormalities of muscle enzymes, electromyography, or muscle biopsy. DM was defined by Bohan and Peter criteria and was 1:1 matched (sex and age ± 5 yrs) to patients with CADM. Anti-MDA5 autoAb levels were determined using ELISA. Clinical features were compared between CADM and DM and between MDA5-positive and MDA5-negative subjects, using chi-squared and/or Mann-Whitney U tests as appropriate.

RESULTS

        : We identified 61 patients with CADM who were matched to 61 DM controls (female 62% vs 64%; mean age 44.8 yrs vs 48.2, p < 0.5). Anti-MDA5 frequency was the same in both cohorts (13.1%), and anti-MDA5 was significantly associated with a higher likelihood of cutaneous ulcers, digital tip ulcerations, and puffy fingers as well as interstitial lung disease (ILD). Most patients with ILD had rapidly progressive ILD (RPILD) leading to early death. Patients with CADM were more likely to have dysphagia, but there were no other clinical differences seen associated with CADM as compared to classic DM.

CONCLUSION

        : Anti-MDA5 positivity had a similar frequency in US patients with CADM and DM and is associated with ILD, RPILD, cutaneous ulcers, digital tip ulceration, and poor survival.

        引自:MOGHADAM-KIA, Siamak, et al. Antimelanoma Differentiation-associated Gene 5 Antibody: Expanding the Clinical Spectrum in North American Patients with Dermatomyositis. The Journal of Rheumatology, 2017, 44.3: 319-325.

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