抗合成酶综合征16例临床特点分析

Clinical characteristics of sixteen cases of antisynthase syndrome

目的分析抗合成酶综合征(antisynthetase syndrome,ASS)的临床表现、诊断和治疗,提高临床医生对ASS的认识。方法回顾性分析中南大学湘雅医学院附属常德医院(常德市第一人民医院)收治的16例ASS患者的临床资料,总结ASS的临床特点、治疗和预后。结果16例患者中女性11名、男性5名,主要症状为气促。抗组氨酰tRNA合成酶(Jo-1)抗体阳性10例,抗苏氨酰tRNA合成酶(PL-7)抗体阳性2例,抗甘氨酰tRNA合成酶(EJ)抗体阳性2例,抗Jo-1抗体、抗亮氨酰tRNA合成酶(OJ)抗体同时阳性1例,抗天冬酰胺基tRNA合成酶(KS)抗体、抗PL-7抗体、抗丙氨酰tRNA合成酶(PL-12)抗体同时阳性1例。肺泡灌洗液细胞学分类检查以中性细胞、嗜酸细胞升高为主,肺功能提示不同程度限制性通气功能障碍及不同程度弥散功能下降。胸部CT主要表现为双下肺胸膜下分布的蜂窝影、网格状影、磨玻璃影及实变影,2名患者合并胸腔积液。治疗上予以糖皮质激素及环磷酰胺治疗,疗效良好。结论ASS主要表现为气促、发热、关节疼痛,血清抗氨基酰tRNA合成酶(anti-aminoacyl tRNA synthetase,ARS)抗体呈阳性。临床医生应加强对ASS的认识,对存在发热、气促、关节疼痛、雷诺现象、技工手的患者,应考虑ASS的诊断,尽早筛查抗ARS抗体。

Objective To analyze the clinical manifestation,diagnosis and treatment of antisynthetase syndrome(ASS)and improve clinicians'understanding of ASS.Methods The clinical data of sixteen ASS patients admitted to Changde Hospital Affiliated to Xiangya School of Medicine,Central South University(The First People's Hospital of Changde City)were analyzed retrospectively,and the clinical characteristics,treatment and prognosis of the disease were summarized.Results Among the 16 patients,11 were female and 5 were males,with the main symptom being shortness of breath.Of the antisynthetase antibodies,there were 10 cases with positive anti-histidine tRNA synthetase(Jo-1)antibody,2 cases with positive antithreonyl tRNA synthetase(PL-7)antibody,2 cases with positive anti-glycyl tRNA synthetase(EJ)antibody,1 case with positive anti-Jo-1 antibody and positive anti-leucyl tRNA synthetase(OJ)antibody,and 1 case with positive anti-asparaginyl tRNA synthetase(KS)antibody,positive anti-PL-7 antibody and positive anti-alanyl tRNA synthetase(PL-12)antibody.Cytologic classification test through alveolar lavage fluid mainly showed that neutrophils and eosinophils increased.Pulmonary function indicated different degrees of restrictive ventilatory dysfunction and decreased diffusing capacity.The chest CT is mainly manifested as honeycomb shadow,grid shadow,ground glass opacities or consolidation and predominantly in the subpleural with bilateral lower lungs predilection.Two patients also had pleural effusion.Glucocorticoid and cyclophosphamide were used in the treatment,and a certain effect was achieved.Conclusion The main symptoms of ASS are shortness of breath,fever and joint pain.Serum anti-aminoacyl tRNA synthetase(ARS)antibody is positive.Clinicians should strengthen the understanding of ASS,and take the diagnosis of ASS into account for patients with fever,shortness of breath,polyarthritis,Raynaud's phenomenon and mechanician hands,so as to check anti-ARS antibody as early as possible.