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Title: [The clinical significance of myositis-specific antibodies in polymyositis/dermatomyositis associated interstitial lung diseases].
Author: Xie MM, Yan X, Li H, Ding JJ, Gui XH, Liu Y, Wang YS, Chen ZY, Cai HR.
Journal: Zhonghua Jie He He Hu Xi Za Zhi; 2018 Aug 12; 41(8):616-621. PubMed ID: 30138971.
Abstract:
Objective: To investigate the profile and clinical significance of myositis-specific antibody spectrum (MSAs) in patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Methods: Sera from 74 patients with PM/DM-ILD, 29 patients with SLE and 32 healthy controls were collected and Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs . The clinical data of all patients were collected from medical records. Statistical analysis was performed using One-way ANOVA, t-test, rank sum test, χ(2) test or Fisher's exact test. Results: The overall detection rate of MSAs in 74 patients with PM/DM-ILD was 86.5%, significantly higher than that in patients with SLE and healthy controls (χ(2)=66.24, 69.85, P<0.01). According to the diagnostic criteria of PM/DM, 18 of 74 patients were definitely diagnosed, 11 were preliminarily diagnosed and 45 were suspected, in which the detection rate of MSA was 83.3%, 90.9% and 86.7%, respectively .The detection rates of MSAs in 17 PM-ILD and 57 DM-ILD were 82.4% and 87.7% respectively. The anti-ARS and anti-MDA5 were the two most common subtypes of MSAs in patients with PM/DM-ILD, the positive rates being 59.5% and 25.7%, respectively . The incidence of CADM, acute/subacute ILD and 90-day mortality in the anti-MDA5 positive group (χ(2)=12.945, 23.203, 26.434, P<0.05) was significantly higher than those of the anti-ARS group and the MSA-negative group, while the incidence of helitrope rash, V-rash, fever was significantly higher than the anti-ARS positive group (χ(2)=11.462, 5.895, 10.609, P<0.05). The incidence of muscle weakness in anti-Jo-1 group was significantly higher than that in the non-Jo-1 antibody group (χ(2)=3.991, P<0.05), while other clinical features were not statistically significant between the anti-Jo-1 and the non-Jo-1 anti-ARS positive groups (P>0.05). Conclusion: The detection rate and accuracy of MSAs in polymyositis/dermatomyositis with ILD was very high, which was useful for early diagnosis of the disease, and severity and prognosis assessment. It is strongly recommended that MSAs should be detected in patients with clinical suspicion of PM/DM-associated interstitial lung diseases. 目的： 观察多发性肌炎/皮肌炎相关间质性肺疾病(PM/DM-ILD)肌炎特异性抗体(MSAs)的分布，分析其临床意义。 方法： 回顾性收集2016年12月至2017年12月在南京大学医学院附属鼓楼医院呼吸科住院的74例PM/DM-ILD患者的血清及临床资料(PM/DM-ILD组)，其中男33例，女41例，年龄30～77岁，平均(54±11)岁，病程0.5～240个月，平均(11±30)个月。同期南京大学医学院附属鼓楼医院风湿免疫科住院的系统性红斑狼疮(SLE)患者29例，其中男5例，女24例，年龄14～56岁，平均(32±13)岁。健康对照组32名，来自南京大学医学院附属鼓楼医院体检中心，其中男10名，女22名。年龄21～79岁，平均(42±17)岁。比较各组MSAs相关检测结果及临床资料，采用单因素方差分析、t检验、秩和检验、χ(2)检验或Fisher′s精确概率法进行统计学分析。 结果： 74例PM/DM-ILD患者中，MSAs阳性患者64例，总体检出率为86%(64/74)，明显高于SLE组及健康对照组(χ(2)＝66.24、69.85；P<0.01)74例患者中18例确诊(18/74，24%)，11例拟诊(11/74，15%)，45例疑诊(45/74，61%)，MSA检出率分别为15例(15/18，83%)、10例(10/11，91%)和39例(39/45，87%)。抗-ARS抗体和抗-MDA5抗体是PM/DM -ILD患者最常检出的2种MSAs抗体，阳性率分别为59.5 %、25.7%。抗MDA5阳性组患者的CADM、急性/亚急性ILD的发生率及90 d病死率显著高于抗ARS组及MSA阴性组(χ(2)＝12.945、23.203、26.434，均P<0.05)，向阳疹、V字疹及发热的发生率明显高于抗ARS阳性组(χ(2)＝11.462、5.895、10.609，均P<0.05)。抗ARS阳性组中，抗Jo-1阳性患者的肌无力发生率显著高于非Jo-1抗体阳性，差异有统计学意义(χ(2)＝3.991，P<0.05)，其他临床特点差异无统计学意义(P>0.05)。 结论： 在PM/DM-ILD患者中，MSAs检出率及准确性很高，有助于疾病早期诊断，严重程度以及预后预测。对临床疑诊为PM/DM相关ILD患者建议进行MSAs检测。.

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