5例抗丙氨酰tRNA合成酶抗体阳性患者临床特征

Clinical Characters of Anti-alanyl-tRNA Synthetase Antibody

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。

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.