Objective: To describe the clinical and radiological characteristics of antisynthetase syndrome associated interstitial lung disease in patients with different serum anti-aminoacyl-tRNA synthetase antibodies. Methods: We conducted a retrospective analysis of 5 adult patients with antisynthetase syndrome associated interstitial lung disease in Peking Union Medical College Hospital. Their clinical and chest radiological data were analyzed and relevant literatures were reviewed. Results: Among these 5 patients, there were 1 male and 4 females, aged from 32 y to 67 y, with a mean age of 53 y. Cough and exertional dyspnea were the main clinical complaints. Four cases showed mechanic's-like hands, and all of the 5 cases had Velcro rales in the basal lungs. None of them showed clubbing. Creatine kinase was elevated in 1 case with anti-Jo-1 synthetase antibody, and anti-nuclear antibody was positive in 4 cases, with different titers from 1∶80 to 1∶320, and anti-Ro-52 antibody was positive in 3 cases. Anti-aminoacyl-tRNA synthetase antibody spectrum analysis showed 1 case with anti-Jo-1, 1 anti-PL-7, 1 anti PL-12, 1 anti-EJ and 1 anti-OJ synthetase antibody, respectively. Chest high resolution CT showed nonspecific interstitial pneumonia pattern in 1 case, and nonspecific interstitial pneumonia pattern with organizing pneumonia pattern in 4 cases. All the cases responded to immunosuppressive therapy including corticosteroids (with starting prednisone dosage more than 1 mg·kg(-1)·d(-1)) plus azathioprine or Mycophenolate mofetil. Conclusions: Antisynthetase syndrome associated interstitial lung disease, characterized by the presence of different anti-tRNA synthetase antibodies, is an increasingly recognized clinical entity. Clinical and radiological features of different subtypes of antisynthetase syndrome are relatively heterogeneous. Nonspecific interstitial pneumonia pattern, nonspecific interstitial pneumonia pattern with organizing pneumonia pattern and organizing pneumonia pattern were common chest HRCT patterns. Prednisone plus immunosuppressive agents are the recommended first line therapy.
目的： 分析常见的不同亚型的抗合成酶综合征相关性间质性肺疾病的临床及影像学表现。 方法： 回顾北京协和医院呼吸内科2015年3月至2017年3月住院的5例不同临床亚型的抗合成酶综合征相关性间质性肺疾病患者，其中男1例、女4例，年龄32～67岁，平均年龄53岁。分析其临床及胸部高分辨率CT的主要特点。 结果： 5例患者均表现为咳嗽、活动后气短，均存在明确的双下肺为主的爆裂音，4例存在"技工手" ，均无"杵状指" 。1例血清肌酸激酶升高，4例抗核抗体阳性(1∶80～1∶640)，3例RO-52阳性；肌炎抗体谱抗Jo-1、抗PL-7、抗PL-12、抗EJ及抗OJ阳性各1例。1例胸部高分辨率CT表现符合非特异性间质性肺炎，4例表现为非特异性间质性肺炎合并机化性肺炎。所有患者接受起始剂量为≥1 mg·kg(－1)·d(－1)泼尼松联合免疫抑制剂治疗，其中2例使用硫唑嘌呤，3例使用吗替麦考酚酯，所有患者治疗后临床症状和胸部CT表现均明显改善。 结论： 抗合成酶综合征相关性间质性肺疾病逐渐被临床认识，不同亚型的抗合成酶综合征的临床表现和胸部影像学表现可能存在差异，以非特异性间质性肺疾病、非特异性间质性肺炎合并机化性肺炎或机化性肺炎多见，治疗推荐使用糖皮质激素联合免疫抑制剂作为初始治疗。.
Keywords: Anti-aminoacyl-tRNA synthetase antibody; Antisynthetase syndrome; Lung disease, interstitial.