抗Jo-1抗体综合征合并间质性肺病患者临床分析

Clinical analysis of anti-Jo-1 antibody syndrome complicated with interstitial lung disease

目的抗Jo-1抗体综合征是以抗Jo-1抗体阳性为特征的抗合成酶抗体综合征,间质性肺病是抗Jo-1抗体综合征常见并且严重的临床表现之一,临床预后不佳。本研究回顾性分析抗Jo-1抗体综合征合并间质性肺病患者的临床资料,并总结临床特点以加深对该疾病的认识,更好地指导临床实践。方法回顾性分析在2016年1月1日至2018年3月31日期间就诊于南京大学医学院附属鼓楼医院呼吸与危重症医学科的抗Jo-1抗体综合征合并间质性肺病患者的临床资料。记录患者的一般情况、临床表现、影像学特征、肺功能参数、实验室检查及治疗方案等资料。根据疾病进展情况将患者分为两组:快速进展型和缓慢进展型。通过T检验、秩和检验、卡方检验等比较两组患者的差异。所有数据均采用SPSS 25.0软件及Graphpad Prism 8软件进行统计分析。P<0.05为差异有统计学意义。结果本研究共纳入65例抗Jo-1抗体综合征合并间质性肺病患者,其中男性29例(44.62%),女性36例(55.38%),平均年龄(57.52±10.92)岁,快速进展型有21例(32.31%),缓慢进展型有44例(67.69%),截止随访日期死亡患者共4例,多数有咳嗽(95.38%)、咳痰(63.08%)、气短(90.77%)等症状,81.54%患者肺部可闻及Velcro音或捻发音或爆裂音,影像学表现多为非特异性间质型肺炎(50.76%)。与缓慢进展型组患者相比,抗Jo-1抗体综合征合并快速进展型间质性肺病组患者更易出现关节肿痛(P<0.001),肌酸激酶、乳酸脱氢酶及血沉更高(P<0.05),动脉血氧分压更低(P<0.001)。结论抗Jo-1抗体综合征合并快速进展型间质性肺病患者更易出现关节肿痛,肌酸激酶、乳酸脱氢酶更高,血沉更快,动脉血氧分压更低,需尽早治疗。

Objective Anti-Jo-1 antibody syndrome is an anti-synthase antibody syndrome characterized by positive anti-Jo-1 antibody.Interstitial lung disease(ILD)is one of the common and serious complications of anti-Jo-1 antibody syndrome,which makes the whole prognosis poor.To retrospectively analyze the clinical data of patients with anti-Jo-1 antibody syndrome complicated with ILD,and to summarize the clinical characteristics so as to deepen the understanding of this kind of disease and guide clinical practice.Methods The clinical data of patients with anti-Jo-1 antibody syndrome complicated with ILD who were admitted to the Department of Respiratory and Critical Care Medicine,Nanjing Drum Tower Hospital Affiliated to Nanjing University Medical School from January 1,2016 to March 31,2018 were retrospectively analyzed.Their general data,clinical manifestations,imaging features,lung function parameters,laboratory tests and treatment were recorded.They were divided into two groups based on the progression of the disease:the rapidly progressive group and the slowly progressive group.T test,rank sum test and Chi-square test were used to compare the differences between the two groups.All data were statistically analyzed by software of SPSS25.0 and GraphpadPrism8.P<0.05 was considered statistically significant.Results A total of 65 patients with anti-Jo-1 antibody syndrome complicated with interstitial lung disease were included in this study,including 29 males(44.62%)and 36 females(55.38%),with an average age of 57.52±10.92 years old.There were 21(32.31%)patients with rapid progression and 44(67.69%)patients with slow progression and 4 patients died in the end of follow-up time.Most of the patients had cough(95.38%),sputum(63.08%),shortness of breath(90.77%)and other symptoms.Lung crepitus could be heard in 81.54%of the patients.The imaging findings of 50.76%patients were non-specific interstitial pneumonia.Compared with patients with slowly progressive interstitial lung disease,patients with rapidly progressive interstitial lung disease were more likely to have pain in the joints(P<0.001),higher creatine kinase,lactate dehydrogenase and erythrocyte sedimentation rate(P<0.05,respectively),and lower arterial partial oxygen pressure(P<0.001).Conclusion The patients with anti-Jo-1 antibody syndrome complicated with rapidly-progressive interstitial lung disease are more likely to have joint swelling and pain,higher creatine kinase,lactate dehydrogenase and sedimentation rate,and lower arterial partial oxygen pressure,requiring early treatment.