Abstract：
Objective: To provide reference for clinicians in diagnosis and treatment of antisynthetase syndrome with interstitial pulmonary disease (ASS-ILD) by analyzing the clinical features, imaging features and pulmonary function changes of ASS-ILD patients. Methods: A total of 92 patients with ASS-ILD diagnosed in the Respiratory Center of China-Japan Friendship Hospital from January 2015 to May 2018 were included, clinical manifestations, high-resolution computed tomography (HRCT), pulmonary function test, treatment and outcome were retrospectively analyzed. Results: The average age of the 92 patients was (58.6±12.2) years with a ratio of male to female 1∶1.79. The main types of anti-synthetase antibody were anti-Jo-1 antibody (37 cases, 40.2%) and anti EJ antibody (26 cases, 28.3%). The most common symptoms of ASS-ILD were cough (79 cases, 85.9%), shortness of breath (60 cases, 65.2%), expectoration (54 cases, 58.7%), fever (34 cases, 36.9%), and common signs were craftsman\'s hand (30 cases, 32.6%) and joint pain (23 cases, 25.0%). The most common imaging findings in HRCT were ground-glass opacities(68 cases, 73.9%), reticulations (45 cases, 48.9%), tractive bronchiectasis (40 cases, 43.5%) and consolidation (39 cases, 42.4%). The most common types of ILD were non-specific interstitial pneumonia (NSIP) (63 cases, 68.5%), followed by NSIP-organic pneumonia (OP) (12 cases, 13.0%). The main type of lung function impairment was mild restrictive ventilation dysfunction. There were no significant differences in clinical features, imaging findings and pulmonary function changes in different subtypes of ASS-ILD patients (all P>0.05). Corticosteroids was used in 78 (84.8%) of ASS-ILD patients, and 34 cases (37.0%) were given cyclophosphamide. A total of 91 patients (98.9%) were discharged with improvement and 1 anti-EJ positive patient died. Conclusions: ASS-ILD occurs frequently in middle-aged and old women. Respiratory symptoms, craftsman\'s hands and arthritis are the most common clinical manifestations. The most common imaging types of ILD are NSIP and NSIP-OP. Corticosteroids is commonly used for treatment.
目的： 总结抗合成酶综合征（ASS）合并间质性肺病（ILD）患者的临床特征。 方法： 回顾性分析2015年1月至2018年5月中日友好医院呼吸中心诊断为ASS-ILD患者92例，对其初诊时的临床表现、高分辨率CT（HRCT）影像学特点、肺功能指标以及治疗与转归进行总结分析。 结果： ASS-ILD患者年龄（58.6±12.2）岁（范围：17~83岁），男女比为1∶1.79，抗合成酶抗体分型以抗Jo-1抗体阳性（37例，40.2%）和抗EJ抗体阳性（26例，28.3%）为主。ASS-ILD最常见的症状是咳嗽（79例，85.9%）、气短（60例，65.2%）、咳痰（54例，58.7%）、发热（34例，36.9%），体征常见技工手（30例，32.6%）及关节疼痛（23例，25.0%）。CT影像学上以磨玻璃影（68例，73.9%）、网格影（45例，48.9%）、牵拉性支气管扩张（40例，43.5%）以及实变（39例，42.4%）常见，ILD类型以非特异性间质性肺炎（NSIP）（63例，68.5%）最多见，其次是NSIP-机化性肺炎（OP）（12例，13.0%）。肺功能改变主要为轻度限制性通气功能障碍。不同抗合成酶抗体亚型的ASS-ILD患者之间临床、影像学及肺功能比较差异均无统计学意义（P值均>0.05）。78例（84.8%）患者应用激素治疗，34例（37.0%）使用环磷酰胺。91例（98.9%）患者好转出院，1例抗EJ阳性患者因呼吸衰竭死亡。 结论： ASS-ILD以中老年女性多发，临床表现以呼吸道症状、技工手、关节炎常见，影像学最常见的类型为NSIP和NSIP-OP，临床治疗常应用激素。.
目的： 总结抗合成酶综合征（ASS）合并间质性肺病（ILD）患者的临床特征。 方法： 回顾性分析2015年1月至2018年5月中日友好医院呼吸中心诊断为ASS-ILD患者92例，对其初诊时的临床表现、高分辨率CT（HRCT）影像学特点、肺功能指标以及治疗与转归进行总结分析。 结果： ASS-ILD患者年龄（58.6±12.2）岁（范围：17~83岁），男女比为1∶1.79，抗合成酶抗体分型以抗Jo-1抗体阳性（37例，40.2%）和抗EJ抗体阳性（26例，28.3%）为主。ASS-ILD最常见的症状是咳嗽（79例，85.9%）、气短（60例，65.2%）、咳痰（54例，58.7%）、发热（34例，36.9%），体征常见技工手（30例，32.6%）及关节疼痛（23例，25.0%）。CT影像学上以磨玻璃影（68例，73.9%）、网格影（45例，48.9%）、牵拉性支气管扩张（40例，43.5%）以及实变（39例，42.4%）常见，ILD类型以非特异性间质性肺炎（NSIP）（63例，68.5%）最多见，其次是NSIP-机化性肺炎（OP）（12例，13.0%）。肺功能改变主要为轻度限制性通气功能障碍。不同抗合成酶抗体亚型的ASS-ILD患者之间临床、影像学及肺功能比较差异均无统计学意义（P值均>0.05）。78例（84.8%）患者应用激素治疗，34例（37.0%）使用环磷酰胺。91例（98.9%）患者好转出院，1例抗EJ阳性患者因呼吸衰竭死亡。 结论： ASS-ILD以中老年女性多发，临床表现以呼吸道症状、技工手、关节炎常见，影像学最常见的类型为NSIP和NSIP-OP，临床治疗常应用激素。.
摘要：
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