Kishaba Tomoo, McGill Rita, Nei Yuichiro, Ibuki Sachi, Momose Masashi, Nishiyama kenta, Nagano Hiroaki, Yamashiro Shin
Department of Respiratory Medicine, Okinawa Chubu Hospital.
Division of Nephrology, University of Chicago.
J Med Invest. 2018;65(3.4):251-257. doi: 10.2152/jmi.65.251.
Dermatomyositis (DM) and polymyositis (PM) often have association with interstitial lung disease (ILD) which have disease specific autoantibody.
We reviewed medical records of DM/PM associated ILD from January 2000 to December 2017 according to the autoantibody.
We identified 52 patients, of whom 30 were antibody negative, 18 had anti aminoacyl-tRNA synthetases (ARS) antibodies and 4 had anti melanoma differentiation-associated gene (MDA)-5 antibody. In high resolution computed tomography (HRCT) of the chest, area of ground glass opacity (GGO), consolidation, and lung tip consolidation were more extensive in anti MDA-5 antibody positive patients (p=0.051, p=0.026, and p=0.027, respectively). Among laboratory findings, GOT had strong correlations with CPK (r=0.889, p < 0.001), and LDH (r=0.910, p < 0.001). Among roentgenographic findings, there were moderate correlations between GGO and consolidation (r=0.668, p < 0.001), and between reticular shadow and traction bronchiectasis (p=0.633, p < 0.001). ILD patients with anti MDA-5 antibodies had decreased survival (1.00 vs 84.3, 22.9 months, p < 0.001).
ILD patients with anti ARS antibody had intense inflammation, but reversible fibrosis and good prognosis. On the other hand, anti MDA-5 antibody positive ILD patients had shorter survival. Extent of parenchymal shadow and serum GOT were useful indicator of disease activity of PM/DM associated ILD patients in our cohort. J. Med. Invest. 65:251-257, August, 2018.
皮肌炎（DM）和多发性肌炎（PM）常与具有疾病特异性自身抗体的间质性肺疾病（ILD）相关。
我们根据自身抗体回顾了2000年1月至2017年12月期间DM/PM相关ILD的病历。
我们确定了52例患者，其中30例抗体阴性，18例有抗氨酰tRNA合成酶（ARS）抗体，4例有抗黑色素瘤分化相关基因（MDA）-5抗体。在胸部高分辨率计算机断层扫描（HRCT）中，抗MDA-5抗体阳性患者的磨玻璃影（GGO）、实变和肺尖实变面积更广泛（分别为p = 0.051、p = 0.026和p = 0.027）。在实验室检查结果中，谷草转氨酶（GOT）与肌酸磷酸激酶（CPK）（r = 0.889，p < 0.001）和乳酸脱氢酶（LDH）（r = 0.910，p < 0.001）有很强的相关性。在影像学检查结果中，GGO与实变之间（r = 0.668，p < 0.001）以及网状阴影与牵拉性支气管扩张之间（p = 0.633，p < 0.001）有中度相关性。抗MDA-5抗体的ILD患者生存率降低（1.00对84.3，22.9个月，p < 0.001）。
抗ARS抗体的ILD患者有强烈炎症，但纤维化可逆且预后良好。另一方面，抗MDA-5抗体阳性的ILD患者生存期较短。在我们的队列中，实质阴影范围和血清GOT是PM/DM相关ILD患者疾病活动的有用指标。《医学调查杂志》65:251 - 257，2018年8月。
