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[抗合成酶相关间质性肺疾病患者的临床及病理表现]

[Clinical and pathological findings of patients with interstitial lung disease associated with antisynthetase].

作者信息

Hara Hiromichi, Inoue Yasushi, Sato Tetsuo

机构信息

Division of Respiratory Diseases, Department of Internal Medicine The Jikei University School of Medicine.

出版信息

Nihon Kokyuki Gakkai Zasshi. 2005 Nov;43(11):652-63.

PMID:16366363
Abstract

Autoantibodies against aminoacyl-tRNA synthetase (ARS) are known to associate with myositis, arthritis, and interstitial lung disease (ILD). Anti Jo-1 antibody is a common diagnostic tool of this syndrome and is available on a commercial basis, while other anti-ARS antibodies such as anti-EJ, anti-PL7 and anti-OJ antibodies are less commonly examined. The aim of this study was to investigate the clinicopathological characteristics of 9 ILD patients who were positive for anti-ARS antibodies including anti-EJ, anti-PL7 and anti-Jo-1 antibodies. In physical examination, muscle pain and/or skin rash (including mechanic's hand) were observed frequently. In laboratory findings, levels of myogenic enzymes were slightly elevated, and anti-nucleus antibody was detected in most cases. Air-space consolidation with traction bronchiectasis, ground glass opacities and characteristic volume loss of both lower lobes were observed in radiological examination. Organization in alveolar spaces and lymphoid alveolitis that did not destroy existing pulmonary structures were main pathological findings. Immunosupressive therapy was effective and the prognosis appears satisfactory in these cases. Although anti-ARS antibodies had been measured as a marker of myositis, we suggest that they are useful as markers of interstitial pneumonia.

摘要

已知抗氨酰-tRNA合成酶(ARS)自身抗体与肌炎、关节炎和间质性肺病(ILD)相关。抗Jo-1抗体是该综合征常见的诊断工具,有商业化产品可供使用,而其他抗ARS抗体如抗EJ、抗PL7和抗OJ抗体则较少被检测。本研究的目的是调查9例抗ARS抗体阳性的ILD患者的临床病理特征,这些抗体包括抗EJ、抗PL7和抗Jo-1抗体。在体格检查中,经常观察到肌肉疼痛和/或皮疹(包括技工手)。在实验室检查结果中,肌源性酶水平略有升高,大多数病例检测到抗核抗体。在放射学检查中,观察到气腔实变伴牵引性支气管扩张、磨玻璃影以及双下叶特征性容积缩小。肺泡腔内机化和未破坏现有肺结构的淋巴性肺泡炎是主要病理表现。免疫抑制治疗有效,这些病例的预后似乎令人满意。尽管抗ARS抗体已被作为肌炎的标志物进行检测,但我们认为它们作为间质性肺炎的标志物很有用。

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引用本文的文献

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Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy.经外科肺活检病理证实的抗甘氨酰tRNA合成酶(抗EJ)抗体相关间质性肺疾病的长期临床病程
BMC Pulm Med. 2016 Dec 1;16(1):168. doi: 10.1186/s12890-016-0325-y.
2
Mechanic's hands revisited: is this sign still useful for diagnosis in patients with lung involvement of collagen vascular diseases?再探机械工之手:此体征对胶原血管病肺部受累患者的诊断仍有用吗?
BMC Res Notes. 2014 May 17;7:303. doi: 10.1186/1756-0500-7-303.