Anti-Mi-2 antibodies characterize a distinct clinical subset of dermatomyositis with favourable prognosis
Anti-Mi-2 antibodies characterize a distinct clinical subset of dermatomyositis with favourable prognosis
复制标题
抗 Mi-2 抗体是皮肌炎的一个独特的临床亚型特征，具有良好的预后
DOI：
10.1684/ejd.2020.3750
复制
发表时间：
2020-03-01
影响因子：
2.5
通讯作者：
Peng, Qing-Lin
中科院分区：
文献类型：
--
作者：
Liang, Lin;Zhang, Ya-Mei;Peng, Qing-Lin
抗Mi-2β抗体是原发性炎症性肌病患者中存在的一种肌炎特异性自身抗体，目的探讨抗Mi-2抗体阳性皮肌炎（DM）患者的临床特点及远期预后。并根据横断面和纵向研究调查了可能相关的临床特征。结果在糖尿病患者中，40/357抗Mi-2β抗体阳性率为11.2%，V征发生率明显高于对照组（72.5% vs 45.7%;p= 0.001），披肩征（60.0% vs 35.6%;p= 0.003）和肌无力（77.5% vs 57.1%;p= 0.013），但间质性肺病（ILD）的发生率较低（37.5% vs 60.9%;p= 0.005）和恶性肿瘤（0% vs 12.0%;p= 0.041）。抗Mi-2β抗体水平与疾病活动性呈正相关。中位随访期44个月后，97.0%的患者显示临床缓解。26例抗Mi-2β阳性患者病程超过2年，16/26例（61.5%）为单周期性无复发。此外，5例（15.1%）患者完全缓解超过3个月。Kaplan-Meier生存曲线显示，与抗Mi-2β阴性患者相比，抗Mi-2β阳性DM患者的死亡率显著降低（对数秩;p= 0.035）。有趣的是，抗Mi-2β抗体并没有随着时间的推移在所有患者中消失。结论抗Mi-2β抗体与ILD和恶性肿瘤发生率低、治疗反应良好和预后良好的DM亚组相关。此外，抗Mi-2β水平与疾病活动相关。
BackgroundAnti-Mi-2 antibody is a type of myositis-specific autoantibody found in idiopathic inflammatory myopathy patients.ObjectivesTo investigate the clinical features and long-term outcomes in anti-Mi-2-positive dermatomyositis (DM) patients.Materials and MethodsSerum anti-Mi-2β antibodies were detected in 357 DM patients by enzyme-linked immunosorbent assays, and possible associated clinical features were investigated based on cross-sectional and longitudinal studies.ResultsOf the DM patients, 40/357 (11.2%) were positive for anti-Mi-2β antibodies and found to have a significantly higher frequency of V sign (72.5% vs 45.7%;p= 0.001), shawl sign (60.0% vs 35.6%;p= 0.003), and muscle weakness (77.5% vs 57.1%;p= 0.013), but a lower incidence of interstitial lung disease (ILD) (37.5% vs 60.9%;p= 0.005) and malignancy (0% vs 12.0%;p= 0.041) than anti-Mi-2β-negative patients. Anti-Mi-2β antibody levels positively correlated with disease activity. After a median follow-up period of 44 months, 97.0% of patients showed clinical remission. Twenty-six anti-Mi-2β-positive patients had a disease course longer than two years, and 16/26 (61.5%) were monocyclic without relapse. Moreover, five patients (15.1%) were drug-free with complete remission for more than three months. Kaplan-Meier survival curves showed that DM patients with positive anti-Mi-2β had a significantly lower mortality rate compared to anti-Mi-2β-negative patients (log-rank;p= 0.035). Interestingly, anti-Mi-2β antibodies did not disappear in all patients over time.ConclusionAnti-Mi-2β antibodies were associated with a subgroup of DM with a low frequency of ILD and malignancy, good treatment response, and favourable outcome. Moreover, anti-Mi-2β levels correlated with disease activity.