Clinical characteristics of patients with anti-aminoacyl-tRNA synthetase antibody positive idiopathic interstitial pneumonia

Clinical characteristics of patients with anti-aminoacyl-tRNA synthetase antibody positive idiopathic interstitial pneumonia
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DOI:
10.1016/j.rmed.2017.10.020
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发表时间:
2017-11-01
影响因子:
4.3
通讯作者:
Mukae, Hiroshi
Mukae, Hiroshi
中科院分区:
医学3区
文献类型:
--
作者:
Yura, Hirokazu;Sakamoto, Noriho;Mukae, Hiroshi

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工作背景:抗氨酰-tRNA合成酶(ARS)抗体已在多发性肌炎/皮肌炎(PM/DM)患者中检测到,尤其与间质性肺病(ILD)相关。目的:探讨抗ARS抗体阳性的特发性间质性肺炎(IIP)患者的临床特点。方法:将患者分为3组:1)抗ARS抗体阳性的IIP(ARS(+)IIP); 2)无抗ARS抗体的IIP(ARS(-)IIP); 3)抗ARS抗体阳性的PM/DM相关ILD(ARS(+)PM/DMILD)。结果:共纳入18例ARS(+)IIP、284例ARS(-)IIP和20例ARS(+)PM/DM-ILD患者。与ARS(+)PM/DM-ILD组相比,ARS(+)IIP组的年龄明显较大,男性占主导地位,结缔组织病体征的患病率较低,HRCT结果和模式存在差异,KL-6水平较高。支气管肺泡灌洗液(BALF)中的结果显示淋巴细胞增多和较低的CD 4/CD 8比值在两组之间相似。与ARS(-)IIP组相比,ARS(+)IIP组痰液百分比明显降低,机械手患病率明显升高,KL-6水平明显升高,肺功能肺活量百分比明显降低,BALF中CD 4/CD 8比值明显降低。然而,包括HRCT结果和较高KL-6水平在内的一些差异表明,与ARS(+)PM/DM-ILD相比,ARS(+)IIP具有严重的ILD。更多患者的进一步前瞻性研究将阐明抗ARS抗体在IIP中的确切作用。
Background: Anti-aminoacyl-tRNA synthetase (ARS)antibodies have been detected in patients with polymyositis/ dermatomyositis (PM/DM)and are especially correlated with interstitial lung disease (ILD). The aim of this study was to clarify the clinical features of patients with anti-ARS antibody positive idiopathic interstitial pneumonias (IIPs).Methods: Patients were classified into three groups: 1) IIP with anti-ARS antibodies (ARS(+)IIP), 2)IIP without anti-ARS antibodies (ARS(-)IIP), and 3) PM/DM-associated ILD with anti-ARS antibodies (ARS(+)PM/DMILD). Clinical characteristics were compared retrospectively between the ARS(+)IIP group and the ARS(-)IIP group or ARS(+)PM/DM-ILD group.Results: Eighteen ARS(+)IIP, 284 ARS(-)IIP, and 20 ARS(+)PM/DM-ILD patients were enrolled. The ARS(+) IIP group was significantly older and the male sex was predominant, had a lower prevalence of signs of connective tissue disease, differences in HRCT findings and patterns, and higher KL-6 levels compared to the ARS (+)PM/DM-ILD group. The findings in the bronchoalveolar lavage fluid (BALF)showing lymphocytosis and a lower CD4/CD8 ratio were similar between the two groups. However, the ARS(+)IIP group had significantly lower percentage of sputum, higher prevalence of mechanic's hand, higher KL-6 levels, lower percentage of vital capacity in the pulmonary function test, and lower CD4/CD8 ratio in BALF, compared to the ARS(-)IIP group.Conclusions: The present study demonstrated that features of pulmonary involvement were similar to those in the ARS(+)PM/DM-ILD group; however, some differences including HRCT findings and higher KL-6 levels suggest that ARS(+)IIP has severe ILD compared with ARS(+)PM/DM-ILD. Further prospective studies with a larger number of patients will elucidate the exact role of anti-ARS antibodies in IIPs.