伴有抗氨酰tRNA合成酶抗体的间质性肺疾病的长期预后。
The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies.
作者信息
Tanizawa Kiminobu, Handa Tomohiro, Nakashima Ran, Kubo Takeshi, Hosono Yuji, Watanabe Kizuku, Aihara Kensaku, Ikezoe Kohei, Sokai Akihiko, Nakatsuka Yoshinari, Taguchi Yoshio, Hatta Kazuhiro, Noma Satoshi, Kobashi Yoichiro, Yoshizawa Akihiko, Oga Toru, Hirai Toyohiro, Chin Kazuo, Nagai Sonoko, Izumi Takateru, Mimori Tsuneyo, Mishima Michiaki
机构信息
Department of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
出版信息
Respir Med. 2017 Jun;127:57-64. doi: 10.1016/j.rmed.2017.04.007. Epub 2017 Apr 15.
RATIONALE
Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS.
OBJECTIVES
To elucidate the long-term outcome of ARS-ILD with and without PM/DM and compare it to that of IPF.
METHODS
A two-center retrospective study was conducted. The study population comprised 36 patients with ARS-ILD (8 with PM, 12 with DM, and 16 without myositis throughout the course), 100 patients with IPF without anti-ARS, and 7 patients with NSIP without anti-ARS. The presence of anti-ARS was determined by RNA immunoprecipitation using the sera obtained at the time of diagnosis before specific treatment.
MEASUREMENTS AND MAIN RESULTS
During the observational period (median 49 months; range, 1-114 months), 7 patients with ARS-ILD (19%; 3 with PM, 1 with DM, and 3 without PM/DM) and 51 patients with IPF (51%) died. Patients with ARS-ILD had better overall survival than those with IPF (log-rank test, P < 0.001) and similar survival compared to those with NSIP (log-rank test, P = 0.59). The prognosis for patients with ARS-ILD was similar between those with and without myositis (log-rank test, P = 0.91). At the median follow-up time of 76.5 months, 14 of the 36 patients with ARS-ILD had deteriorated. Both a decline in forced vital capacity or an initiation of long-term oxygen therapy during the course (odds ratio [OR], 5.34) and acute exacerbation (OR, 28.4) significantly increased the mortality risk.
CONCLUSIONS
The long-term outcome of ARS-ILD was significantly better than that of IPF regardless of the presence or absence of myositis.
理论依据
抗氨酰转运RNA合成酶抗体（抗ARS）是一组肌炎特异性自身抗体，在多发性肌炎和皮肌炎（PM/DM）患者的血清中可检测到，在无任何结缔组织病（CTD）的特发性间质性肺炎患者（包括PM/DM患者）的血清中也可检测到。尽管我们报告了伴有和不伴有PM/DM的抗ARS抗体相关性间质性肺病（ARS-ILD）的临床特征，但ARS-ILD的长期预后仍未确定。由于我们之前的研究表明，不伴有PM/DM的ARS-ILD与CTD相关的ILD相似，而伴有PM/DM的ARS-ILD在影像学上提示非特异性间质性肺炎（NSIP）病理模式，我们推测ARS-ILD的预后可能与无抗ARS的特发性肺纤维化（IPF）不同。
目的
阐明伴有和不伴有PM/DM的ARS-ILD的长期结局，并将其与IPF的结局进行比较。
方法
进行了一项双中心回顾性研究。研究人群包括36例ARS-ILD患者（8例伴有PM，12例伴有DM，16例在整个病程中无肌炎）、100例无抗ARS的IPF患者和7例无抗ARS的NSIP患者。在诊断时未进行特异性治疗前采集血清，通过RNA免疫沉淀法确定抗ARS的存在情况。
测量指标和主要结果
在观察期（中位时间49个月；范围1-114个月）内，7例ARS-ILD患者（19%；3例伴有PM，1例伴有DM，3例不伴有PM/DM）和51例IPF患者（51%）死亡。ARS-ILD患者的总生存率优于IPF患者（对数秩检验，P<0.001），与NSIP患者的生存率相似（对数秩检验，P=0.59）。伴有和不伴有肌炎的ARS-ILD患者的预后相似（对数秩检验，P=0.91）。在中位随访时间76.5个月时，36例ARS-ILD患者中有14例病情恶化。病程中用力肺活量下降或开始长期氧疗（比值比[OR]，5.34）以及急性加重（OR，28.4）均显著增加死亡风险。
结论
无论是否伴有肌炎，ARS-ILD的长期结局均显著优于IPF。
Zotero 插件