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Characteristics of Antisynthetase Syndrome with Anti-threonyl tRNA Synthetase Antibody
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摘要:
目的 探讨抗苏氨酰tRNA合成酶(threonyl-tRNA synthetase, PL-7)抗体阳性的抗合成酶综合征(antisynthetase syndrome, ASS)的临床、血清学和影像学特征。 方法 收集2010年8月至2011年12月间北京协和医院诊治的5例抗PL-7抗体阳性ASS住院患者的资料, 总结其临床表现、实验室检查和影像学特征。 结果 5例ASS患者均有肌炎和肺间质病变, 发热4例, 多关节炎2例, 均未见雷诺现象和技工手。胞浆型抗核抗体阳性4例, 抗Ro-52抗体阳性3例。肺功能检测提示限制性通气功能和/或弥散功能障碍。胸部高分辨CT显示双下肺网格影和磨玻璃影, 伴牵拉性支气管扩张及散在实变影。大剂量糖皮质激素联合环磷酰胺和/或甲氨蝶呤治疗取得较好的临床效果。 结论 抗PL-7抗体阳性的ASS以肌炎和肺间质病变为突出特征, 可伴发热和关节炎, 对免疫抑制治疗反应较好。 -
关键词:
- 抗合成酶综合征 /
- 抗苏氨酰tRNA合成酶抗体 /
- 肌炎 /
- 肺间质病变 /
- 高分辨CT
Abstract:Objective To describe the clinical, laboratory, and radiographic characteristics of antisynthetase syndrome (ASS) patients with anti-threonyl tRNA synthetase (anti-PL-7) antibody. Methods The clinical, laboratory, and radiographic data of five ASS cases with positive anti-PL-7 antibody hospitalized in Peking Union Medical College Hospital from August 2010 to December 2011 were retrospectively analyzed. Results All these five patients demonstrated myositis, including symmetrical proximal muscle weakness (4/5), increased serum creatine phosphokinase (5/5), myogenic damage on electromyography (4/5), and positive results on biopsy (3/3). Four patients met the classification criteria for polymyositis (PM), and one patient presented typical rash of dermatomyositis (DM). Four patients had fever at onset, and two patients showed inflammatory arthritis. None of these patients developed Raynaud phenomenon or mechanic hands. Four patients demonstrated a cytoplasmic pattern when testing for antinuclear antibodies by immunofluorescent assay, and all had anti-PL-7 antibodies. Three patients had positive anti-Ro-52 antibodies. All patients had evidence of interstitial lung disease (ILD) by clinical examination, imaging, or pulmonary function testing. Four patients presented dry cough, gradual dyspnea, and crackles at lung base. Three patients presented with a restrictive pulmonary 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 is highly suggestive of ASS-related ILD. Patients were responsive to high-dose corticosteroid plus cyclophosphamide and/or methotrexate. Conclusion Anti-PL-7 antibody is closely associated with PM/DM with ILD, which may be accompanied with fever and arthritis. Treatment with high-dose glucocorticosteroid and immunosuppressants may result in good response. -
表 1 5例抗合成酶综合征患者的临床、实验室和影像学特征
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