Analysis of clinical features of ruccrent interstitial lung disease in patients with anti-EJ positive antisynthetase syndrome
朱玉静 1王磊 1吕成银 1谈文峰 1张缪佳1
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作者信息
1. 南京医科大学第一附属医院风湿免疫科,南京 210029
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摘要
目的:总结 57 例抗 EJ(anti-glycyl tRNA synthetase)抗体阳性抗合成酶综合征(antisynthetase syndrome,ASS)相关间质性肺疾病(interstitial lung disease,ILD)患者的临床特征,并分析ILD复发的相关因素.方法:选择2020年1月1日至2024年6月30日在南京医科大学第一附属医院(江苏省人民医院)风湿免疫科就诊的57例抗EJ抗体阳性ASS患者的病例资料进行回顾性分析,收集的数据包括患者的一般情况、临床表现、实验室检查结果和胸部CT影像,以及肺功能检查结果[包括用力肺活量(forced vital capacity,FVC)、第1秒用力呼气容积(forced expiratory volume in the first second,FEV1)和一氧化碳弥散量(diffusion lung carbon monoxide,DLCO)],并进一步分析ILD复发的临床特征.结果:所有57例抗EJ抗体阳性ASS患者均合并ILD,平均发病年龄为(58.18±10.27)岁,平均病程为3.00(2.00,16.00)个月.患者中70.18％为女性,87.72％有咳嗽症状,70.18％有咳痰症状,89.47％出现呼吸困难,14.04％发生呼吸衰竭.肺功能结果显示,FVC占正常预计值的百分比(FVC％)、FEV1占正常预计值的百分比(FEV1％)和DLCO占正常预计值的百分比(DLCO％)分别为59.36±21.41、58.34±19.46和58.17±27.95,氧合指数为(363.24±99.42)mmHg.胸部CT影像中,非特异性间质性肺炎(nonspecific inter-stitial pneumonia,NSIP)为最常见的影像学表现类型.在46例完成12个月以上随访的患者中,21例(45.65％)出现了ILD复发.复发组的平均发病年龄为(61.38±8.63)岁,而非复发组为(55.28±11.85)岁,差异无统计学意义(P=0.056).进一步分析发现,复发组的红细胞沉降率(erythrocyte sedimentation rate,ESR)水平显著高于非复发组[(50.48±29.64)mm/h vs.30.28±23.97)mm/h,P=0.025],血清 IgM(immune globulin M,IgM)水平也明显高于非复发组(P=0.042).此外,复发组的CD8+T细胞百分比显著高于非复发组(25.48±11.81 vs.18.59±8.53,P=0.027).尽管复发组患者在基线时的年龄偏大,且ESR、IgM和CD8+T细胞百分比在两组间差异有统计学意义,但多因素二元Logistics回归分析显示,这些指标均未被证实为ILD复发的独立危险因素.结论:ILD是抗EJ抗体阳性ASS患者的主要临床表现,并且常伴有显著的肺功能损伤.尽管糖皮质激素和免疫抑制剂的联合治疗对大多数患者有效,但ILD的复发率依然较高.基线ESR水平增快、IgM升高及CD8+T细胞百分比增加的患者更容易出现复发.
Abstract
Objective:To summarize the clinical characteristics of 57 patients diagnosed with anti-glycyl tRNA synthetase(anti-EJ)positive antisynthetase syndrome(ASS),a subtype of anti-glycyl tRNA positive ASS,complicated by interstitial lung disease(ILD),and to investigate the factors asso-ciated with ILD recurrence.Methods:A retrospective analysis was conducted on the clinical data of 57 anti-EJ positive ASS patientswho were treated at the First Affiliated Hospital of Nanjing Medical Universi-ty from January 1,2020 to June 30,2024.The data collected included demographic information,clinical characteristics,laboratory test results,chest CT findings,and pulmonary function tests.The characteris-tics of ILD recurrence were also analyzed.Results:All the 57 patients with anti-EJ positive ASS were diagnosed with ILD.The mean age at disease onset was(58.18±10.27)years,with a mean disease duration of 3.00(2.00,16.00)months.Among the patients,70.18％were female,87.72％experi-enced a cough,70.18％had expectoration,89.47％reported respiratory difficulties,and 14.04％de-veloped respiratory failure.The results of pulmonary function test showed that the percentage of forced vi-tal capacity(FVC)in the normal predicted value(FVC％),the percentage of forced expiratory volume in the first second(FEV1)in the normal predicted value(FEV1％)and the percentage of diffusion lung carbon monoxide(DLCO)in the normal predicted value(DLCO％)were 59.36±21.41,58.34±19.46 and 58.17±27.95,respectively.The oxygenation index was(363.24±99.42)mmHg.Chest CT imaging showed that nonspecific interstitial pneumonia(NSIP)was the most common radiographic pattern.Among the 46 patients who completed a follow-up of more than 12 months,21 cases(45.65％)showed recurrence of ILD.The average age of onset for the recurrence group was(61.38±8.63)years,while that for the non-recurrence group was(55.28±11.85)years,with a difference approaching statis-tical significance(P=0.056).Further analysis showed that the ESR(erythrocyte sedimentation rate)level was significantly higher in the recurrence group than in the non-recurrence group[(50.48±29.64)mm/h vs.30.28±23.97)mm/h,P=0.025],and the IgM(immune globulin M)level was also significantly higher in the recurrence group(P=0.042).Moreover,the CD8+T proportion was significantly higher in the recurrence group than in the non-recurrence group(25.48±11.81 vs.18.59±8.53,P=0.027).Despite the fact that the recurrence group had a higher baseline age,higher ESR,IgM,and CD8+T proportion,multivariate binary logistic regression analysis showed that these indicators were not independent risk factors for ILD recurrence.Conclusion:ILD is the most common clinical manifestation in patients with anti-EJ positive ASS,with a significant impact on pulmonary function.Al-though the patients responded well to a combination of glucocorticoid and immunosuppressive therapies,the recurrence rate remains high,particularly in those with increased sputum production,and elevated ESR.Close monitoring and early intervention for high-risk patients are essential to improving long-term outcomes.
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