CAJ | 학술논문

目的：探讨抗丙氨酰 tRNA 合成酶（alannyl tRNA synthetase，PL-12）抗体阳性的抗合成酶综合征（anti-synthetase syndrome，ASS）患者的临床特征。方法分析2010年8月至2013年8月北京协和医院5例抗 PL-12抗体阳性 ASS 住院患者的临床表现、血清学结果和影像学改变。结果5例抗 PL-12抗体阳性患者的基础疾病为皮肌炎2例，类风湿关节炎/干燥综合征、系统性硬化症和间质性肺炎各1例。5例患者均有肺间质病变，4例为首发和突出表现，胸部高分辨计算机断层扫描显示双下肺网格影和磨玻璃影为主；肺功能提示限制性通气功能和弥散功能障碍。典型皮肌炎皮损和技工手各2例，肌炎、关节炎、雷诺现象和发热各1例。胞浆型抗核抗体阳性4例，抗 Ro-52抗体阳性4例，抗 SSA 抗体阳性1例。5例患者中4例应用大剂量糖皮质激素（0．8～1．5 mg·kg -1·d -1）联合环磷酰胺（100 mg/d），1例还联用了甲氨蝶呤和环孢菌素 A，1例单独应用雷公藤多甙。治疗后3例患者病情好转，2例患者病情稳定。结论抗 PL-12抗体与肺间质病变密切相关，而肌炎少见。
목적：탐토항병안선 tRNA 합성매（alannyl tRNA synthetase，PL-12）항체양성적항합성매종합정（anti-synthetase syndrome，ASS）환자적림상특정。방법분석2010년8월지2013년8월북경협화의원5례항 PL-12항체양성 ASS 주원환자적림상표현、혈청학결과화영상학개변。결과5례항 PL-12항체양성환자적기출질병위피기염2례，류풍습관절염/간조종합정、계통성경화증화간질성폐염각1례。5례환자균유폐간질병변，4례위수발화돌출표현，흉부고분변계산궤단층소묘현시쌍하폐망격영화마파리영위주；폐공능제시한제성통기공능화미산공능장애。전형피기염피손화기공수각2례，기염、관절염、뢰낙현상화발열각1례。포장형항핵항체양성4례，항 Ro-52항체양성4례，항 SSA 항체양성1례。5례환자중4례응용대제량당피질격소（0．8～1．5 mg·kg -1·d -1）연합배린선알（100 mg/d），1례환련용료갑안접령화배포균소 A，1례단독응용뢰공등다대。치료후3례환자병정호전，2례환자병정은정。결론항 PL-12항체여폐간질병변밀절상관，이기염소견。
Objective The purpose of this study was to examine the clinical profile of patients with anti-alanyl-tRNA synthetase (anti-PL-1 2)antibody.Methods Five patients with anti-PL-1 2 antibody admitted into Peking Union Medical College Hospital were presented.The major clinical,serological,and radiographic data were analyzed.Results All 5 patients with anti-PL-1 2 antibody had evidence of interstitial lung disease (ILD)by clinical examination,imaging and pulmonary function tests.Clinical features presented were dry cough,gradual dyspnea,and crackles at lung bases.Lung function test showed a restrictive pulmonary dysfunction pattern and impaired gas exchange.High-resolution computed tomography scan revealed (predominantly basilar)reticular and ground-glass opacities,accompanied by a loss of lung volume,traction bronchiectasis,and scattered (usually peribronchovascular)areas of consolidation.This distinct pattern was highly suggestive of antisynthetase syndrome-related ILD.Four of 5 anti-PL-12 positive patients had underlying connective tissue disease,including dermatomyositis (2 cases),Sjoegren’s syndrome/rheumatoid arthritis (1 case),and systemic sclerosis (1 case).Only one of five anti-PL-12 positive patients demonstrated clinical myositis,including symmetrical proximal muscle weakness,increased serum creatine phosphokinase,myogenic damage on electromyography,and positive results on biopsy.Two patients had typical rash of dermatomyositis (DM).Mechanic hands occurred in 2 patients,fever in 1 patient,Raynaud’ s phenomenon in 1 patient,and inflammatory polyarthritis with anti-cyclic citrullinated polypeptide antibody in 1 patient.Four patients demonstrated a cytoplasmic pattern when tested for antinuclear antibodies by immunofluorescent assay.Four patients had positive anti-Ro-52 antibodies.Four of 5 patients received high-dose glucocorticosteroid (prednisone 0.8 ~1.5 mg·kg -1 ·d -1 )plus cyclophosphamide (100 mg·d -1 ) therapy.Response was favorable with recovery in 3 patients and stable disease in other 2 patients.Conclusion Anti-PL-12 antibody is strongly associated with the presence of ILD,but less so with myositis.