摘要
目的探讨不同病程抗黑色素瘤分化相关基因5(MDA5)抗体阳性皮肌炎(DM)患者的临床特点及合并快速进展型间质性肺疾病(RP-ILD)的早期预测。方法回顾性分析南京医科大学炎性肌病及结缔组织病相关间质性肺病专病联盟所收集的249例抗MDA5抗体阳性DM患者的临床资料, 根据病程长短分为2组, 其中短病程组(≤6个月)207例, 长病程组(>6个月)42例。符合正态分布的计量资料采用t检验, 非正态分布的计量资料采用 Mann-WhitneyU检验, 计数资料采用χ^(2)检验。危险因素分析采用二元Logistic回归, 绘制ROC曲线评估各危险因素的预测价值, 生存分析采用Kaplan-Meier法。结果短病程组患者肌无力、皮疹、Gottron征、V字征、甲周红斑及技工手的发生率高于长病程组, 而向阳疹、披肩征、关节炎的发生率较低, 但差异均无统计学意义(P>0.05), 短病程组患者重度ILD、RP-ILD的发生率明显增高, 且差异有统计学意义(P<0.05)。短病程组(29.9%)病死率明显高于长病程组(4.8%), 差异有统计学意义(P<0.05)。短病程组患者ALT、AST、LDH、CK及C反应蛋白水平及抗Ro52抗体阳性率均高于长病程组, 差异有统计学意义(P<0.05)。两组患者ESR、铁蛋白水平及抗ARS抗体阳性率差异均无统计学意义(P>0.05)。短病程组中, 男性及抗Ro52抗体是抗MDA5抗体阳性DM患者合并RP-ILD的独立危险因素, 而皮疹和关节炎为合并RP-ILD的保护性因素。ROC曲线结果显示, 抗Ro52抗体对短病程组抗MDA5抗体阳性DM患者合并RP-ILD的具有较好的诊断价值, 敏感度为54.62%, 特异度为89.77%, AUC为0.722(P<0.001)。死亡患者共60例, 其中短病程组患者58例, 长病程组患者2例, 短病程组患者生存时间比长病程组更短, 差异有统计学意义(P<0.001)。结论短病程抗MDA5抗体阳性DM患者合并RP-ILD的风险较长病程患者增高, 病死率更高, 生存时间更短, 抗Ro52抗体可能是其预后不良因素。
Objective:To investigate the clinical characteristics and early diagnosis of rapidly progressive interstitial lung disease(RP-ILD)in patients with anti-MDA5-positive dermatomyositis of different disease stages.Methods:The clinical data of 249 patients with anti-MDA5 antibody positive DM collected from Inflammatory myopathy and Connective tissue Disease-Associated Interstitial Lung Disease Alliance of Nanjing Medical University were analyzed retrospectively,of which 207 cases were in the short-duration group(≤6 months),and 42 cases were in the long-duration group(>6 months).The measurement data conforming to the normal distribution should be subjected to t test,the measurement data of non-normal distribution should be subjected to Mann-Whitney U test,and the counting data shoulb be subjected to χ^(2) test.The risk factors were analyzed by binary logistic regression,and the predictive value of each risk factor was evaluated by drawing ROC curve.The survival analysis was performed by Kaplan-Meier method.Results:The incidence of muscle weakness,rash,Gottron′s sign,V sign,perlungual erythematosus and mechanic′s hands was higher in patients in the short course group than in the long course group,whereas the incidence of heliotrope rash,shawl sign,and arthritis was lower(P>0.05).Furthermore the incidence of ILD and RP-ILD in the patients in the short-course group was significantly higher,and the difference was statistically significant(P<0.05).The mortality rate in the short-course group(29.9%)was significantly higher than that in the long-course group(4.8%),and the difference was statistically significant(P<0.05).The levels of ALT,AST,LDH,CK and CRP and the anti-Ro52 positivity were higher in the short-course group than in the long-course group,and the difference was statistically significant(P<0.05).The differences in ESR,ferritin levels and ARS positivity between the two groups were not statistically significant(P>0.05).In the short-course group,male and anti-Ro52 were independent risk factors for RP-ILD in anti-MDA5-positive dermatomyositis patients,while rash and arthritis were protective factors for RP-ILD.The results of the ROC curve showed that the anti-Ro52 had a better diagnostic value for RP-ILD in anti-MDA5-positive dermatomyositis patients in the short-course group,with a sensitivity of 54.62%and a specificity of 89.77%(P<0.001).specificity 89.77%,and an AUC of 0.722(P<0.001).A total of 60 patients died,including 58 patients in the short-course group and 2 patients in the long-course group,and the survival time of patients in the short-course group was shorter than that in the long-course group,with a statistically significant difference(P<0.001).Conclusion:Patients with short-duration anti-MDA5 antibody positive DM have an increased risk of RP-ILD,a higher mortality rate,and a shorter survival time than patients with long-duration disease,Anti-Ro52 may be a poor prognostic factor.
作者
程路
徐凌霄
徐艳
高文佳
于哲
高玲
臧银善
Cheng Lu;Xu Lingxiao;Xu Yan;Gao Wenjia;Yu Zhe;Gao Ling;Zang Yinshan(Department of Rheumatology and Immunology,the Affiliated Suqian First People's Hospital of Nanjing Medical University,Suqian 223800,China;Department of Rheumatology and Immunology,the First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China)
出处
《中华结核和呼吸杂志》
北大核心
2025年第8期726-731,共6页
Chinese Journal of Tuberculosis and Respiratory Diseases
关键词
皮肌炎
病程
抗MDA5抗体
快速进展性间质性肺疾病
Dermatomyositis
Course of disease
Anti-melanoma differentiation-associated gene 5 antibody
Rapidly progressive interstitial lung disease
