46例抗合成酶综合征临床诊疗分析  

Clinical management of antisynthetase syndrome: Series of 46 cases

作　　者：张威[1] 孟耸 谢社玲 王凯飞[1] 孙军平[1] 解立新[1] ZHANG Wei;MENG Song;XIE Sheling;WANG Kaifei;SUN Junping;XIE Lixin(Department of Pulmonary & Critical Care Medicine, the First Medical Center, Chinese PLA General Hospital, Beijing 100853, China)

机构地区：[1]解放军总医院第一医学中心呼吸与危重症医学科,北京100853

出　　处：《解放军医学院学报》2019年第2期145-148,152,共5页Academic Journal of Chinese PLA Medical School

摘　　要：目的总结不同亚型抗合成酶综合征(antisynthetase syndrome,ASS)特点、治疗及预后。方法回顾2010年1月-2018年7月本中心诊断的46例ASS患者临床资料,分析不同亚型ASS表现、实验室检查结果、治疗、转归及预后,并着重分析ASS合并间质性肺疾病(interstitial lung disease,ILD)的特点。结果 46例中,女性35例,男性11例,发病年龄24～86岁,平均53岁,确诊时病程0.5～720个月,平均36个月,抗Jo-1抗体阳性35例(76.1%),抗EJ抗体阳性6例(13.0%),抗PL-7抗体阳性3例(6.5%),抗PL-12抗体阳性1例(2.2%),抗OJ抗体阳性1例(2.2%)。合并ILD 45例(97.8%),以ILD起病23例(50%)。确诊多发性肌炎(polymyositis,PM)、皮肌炎(dermatomyositis,DM)分别为9例(19.6%)、4例(8.7%),见于抗Jo-1抗体、抗PL-7抗体阳性患者;关节炎22例(47.8%),抗Jo-1抗体、抗PL-12抗体阳性患者发生率较高;雷诺现象9例(19.6%),仅见于抗Jo-1抗体阳性患者;技工手20例(43.5%),抗Jo-1抗体、抗OJ抗体阳性患者发生率较高;发热18例(39.1%),见于抗Jo-1抗体、抗EJ抗体、抗PL-7抗体阳性患者。合并肿瘤3例(6.5%),心肌损害(心律失常)3例(6.5%),食管受累(吞咽困难)2例(4.3%)。46例均给予≥1 mg/(kg·d)糖皮质激素口服或静脉治疗,33例给予环磷酰胺静脉治疗,4例给予吗替麦考酚酯口服治疗,4例给予他克莫司口服治疗,25例给予人免疫球蛋白静脉治疗。42例病情好转,4例因呼吸衰竭死亡。结论不同亚型ASS有相同表现,但出现时间及频率不同,且半数以上以ILD起病。糖皮质激素为治疗ASS一线药物,建议联合使用免疫抑制剂,激素治疗无效或病情进展较快者可给予免疫球蛋白治疗。诊断较早、诊断时ILD较轻者预后较好。Objective To summarize the characteristics, treatment and prognosis of different subtypes of antisynthetase syndrome(ASS). Methods Clinical data about 46 patients with antisynthetase syndrome admitted to our center from January 2010 to July2018 were collected, and the clinical manifestations, laboratory test results, treatment, outcomes and prognosis of different subtypes of antisynthetase syndrome were investigated and the characteristics of ASS combined with interstitial lung disease(ILD) were emphasized. Results Of the 46 cases, there were 35 women and 11 men. The age of onset was 24-86 years, and the average age was 53 years. The course of disease was 0.5-720 months(Mean, 36 months). Anti-Jo-1 antibody was found to be positive in 35(76.1%)cases, anti-EJ antibody in 6(13.0%) cases, anti-PL-7 antibody in 3(6.5%) cases, anti-PL-12 antibody in 1(2.2%) case and anti-OJ antibody in 1(2.2%) case. ILD simultaneously occurred in 45 patients(97.8%), and it was the ?rst ?nding in 23 cases(50%). The de?nite diagnosis of polymyositis(PM) was established in 9(19.6%) patients and dermatomyositis(DM) in 4(8.7%) cases, mainly presented in anti-Jo-1 antibody and anti-PL-7 antibody positive patients. Arthritis occurred in 22(47.8%) cases, and it showed higher incidence in anti-Jo-1 and anti-PL-12 antibody positive patients. Raynaud’s phenomenon showed only in 9(19.6%) anti-Jo-1 antibody positive patients. Mechanic’s hands showed in 20(43.5%) cases, and it showed higher incidence in anti-Jo-1 antibody and anti-OJ antibody positive patients. Fever presented in anti-Jo-1 antibody, anti-EJ antibody and anti-PL-7 antibody positive patients(18, 39.1%). Concomitant tumor was found in 3(6.5%) cases, myocardial damage in 3(6.5%) cases and esophageal involvement in2(4.3%) cases. All patients were given glucocorticoids(≥ 1 mg/(kg · d)) orally or intravenously, 33 patients received intravenous cyclophosphamide, 4 patients received oral mycophenolate mofetil, 4 patients received oral tacrolimus and 25 patients received intravenous im

关 键 词：抗合成酶综合征 抗合成酶抗体 间质性肺疾病 

分 类 号：R593.2[医药卫生—内科学]