天津医药

天津医药 ›› 2019, Vol. 47 ›› Issue (3): 269-273.doi: 10.11958/20182151

• 临床研究 • 上一篇    下一篇

抗MDA5抗体阳性皮肌炎患者合并间质性肺疾病的临床预后因素分析

王洁蕊，舒荣，石慧婧，刘白鹭，李娜，杨文芳，胡玉琴，韩依轩   

  1. 河北省唐山市开滦总医院风湿免疫科（邮编063000）
  • 收稿日期:2018-12-29 修回日期:2019-02-13 出版日期:2019-03-15 发布日期:2019-04-24
  • 通讯作者: 王洁蕊 E-mail:feiyue063000@163.com

Evaluation of clinical prognostic factors of interstitial lung disease in patients with anti-MDA5 antibody positive dermatomyositis

WANG Jie-rui,SHU Rong,SHI Hui-jing,LIU Bai-lu,LI Na,YANG Wen-fang,HU Yu-qin,HAN Yi-xuan   

  1. Department of Rheumatology, Kailuan General Hospital, Tangshan 063000, China
  • Received:2018-12-29 Revised:2019-02-13 Published:2019-03-15 Online:2019-04-24
  • Contact: jie-rui wang E-mail:feiyue063000@163.com

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摘要： 目的 回顾性分析抗黑色素瘤分化相关基因 5（MDA5）抗体阳性皮肌炎（DM）患者合并间质性肺疾病（ILD）的临床表现及预后因素。方法 选取2017年1月—2018年4月开滦总医院收治的DM-ILD患者为研究对象：包括9例抗MDA5抗体阳性DM-ILD（4例幸存者、5例死亡）和9例抗合成酶（ARS）抗体阳性DM-ILD，对2组患者的临床表现进行比较。采用校正年龄和性别的 Cox 回归分析确定抗 MDA5 抗体相关 ILD 预后影响因素。结果 与ARS-ILD患者相比，MDA5-ILD患者涎液化糖链抗原（KL-6）、丙氨酸转氨酶（ALT）、谷氨酰转肽酶（GGT）和铁蛋白水平显著增高（P < 0.05）。4例存活患者和5例死亡患者在发病年龄、性别、CADM发生比例、肌酸激酶（CK）、KL-6、乳酸脱氢酶（LDH）、天冬氨酸转氨酶（AST）、ALT、GGT、C反应蛋白（CRP）和铁蛋白水平比较差异均无统计学意义（P >0.05）。在校正混杂因素后，铁蛋白水平与抗MDA5抗体阳性患者的预后不相关。结论 抗MDA5抗体是早期诊断DM合并急性/亚急性间质性肺炎（A/SIP）的重要指标。尚不能证明铁蛋白水平与抗MDA5抗体阳性ILD的疾病严重程度和预后有关。

关键词: 皮肌炎, 肺疾病, 间质性, 黑色素瘤分化相关基因5, 铁蛋白, 巨噬细胞活化

Abstract: Objective To analyze retrospectively clinical manifestations and prognostic factors of interstitial lung disease (ILD) in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis(DM). Methods The patients with DM-ILD admitted to Kailuan General Hospital between January 2017 and April 2018 were selected as subjects including 9 patients with anti-MDA5 antibody positive (4 survivors，5 deaths) and 9 patients with anti-synthase (ARS)-antibody positive. The clinical manifestations were compared between the two groups. Cox regression analysis adjusted for age and sex was used to determine prognostic factors for anti-MDA5 antibody-related ILD. Results Compared with ARS-ILD patients, krebs von den lungen 6 (KL-6), alanine aminotransferase (ALT), glutamyltransferase (GGT) and ferritin levels were significantly higher in MDA5-ILD patients (P＜0.05). There were no significant differences in age, sex, incidence of CADM, creatine kinase (CK), KL-6, lactate dehydrogenase (LDH), aspartate aminotransferase (AST),ALT, GGT, C-reactive protein (CRP) and ferritin levels between 4 survivors and 5 dead patients (all P＞0.05). After adjusting confounding factors, the level of ferritin was not associated with the prognosis of anti-MDA5 antibody positive patients. Conclusion Anti-MDA5 antibody is an important index for early diagnosis of DM complicated with acute /subacute interstitial pneumonia (A / SIP). It has not yet been demonstrated that the level of ferritin is associated with the severity of disease and prognosis of anti-MDA5 antibody-related ILD.

Key words: dermatomyositis, interstitial lung disease, melanoma differentiation related gene 5, ferritin, macrophage activationactivation