CAJ | 학술논문

目的检测特发性炎性肌病(IIM)患者血清中抗核基质蛋白2(NXP-2)抗体水平,探讨该抗体阳性的IIM患者的临床和实验室特征.方法选取我院收治的IIM患者198例,其中幼年型皮肌炎(JDM)15例,DM 133例,PM 50例.其他CTD包括SLE 70例,RA 60例,SSc 15例,pSS 46例,MCTD 10例和健康对照60名.采用ELISA法测定上述人群血清中抗NXP-2抗体,抗体阳性血清采用免疫沉淀法进一步验证.采用t检验、Mann-Wittney U检验和x2检验等进行统计学分析.结果血清抗NXP-2抗体在IIM中的阳性率为5.1％(10/198),其中JDM组阳性率为20.0％(3/15),DM组3.7％(5/133),PM组4.0％(2/50),3组间差异有统计学意义(P＜0.05).而在其他CTD组及健康对照组的阳性率均为0.抗NXP-2抗体阳性的IIM患者发病年龄[(33±20)岁]低于抗体阴性组[(45±17)岁](t=-2.09,P＜0.05),皮下钙质沉积的发生率[30.0％(3/10)]高于抗体阴性组[2.6％(5/188)] (x2=0.7,P＜0.01),而2组间性别、病程、关节炎、皮疹、近端肌无力、肌痛、吞咽困难、合并间质性肺病和肿瘤等的发生率差异无统计学意义.随访分析,10例抗体阳性的IIM患者中,3例为JDM,其中1例JDM在随访10个月后死亡,该患儿血清抗NXP-2抗体滴度明显升高,合并广泛的皮下钙质沉积,严重的肌无力和快速进展的病情.结论本研究首次对我国IIM及其他CTD患者血清中抗NXP-2抗体进行检测,发现抗NXP-2抗体是IIM的特异性抗体,该抗体阳性者具有独特的临床表型.开展该抗体的检查对IIM诊断有重要价值,而且对IIM的临床分型有重要的指导意义.
목적 검측특발성염성기병(IIM)환자혈청중항핵기질단백2(NXP-2)항체수평,탐토해항체양성적IIM환자적림상화실험실특정.방법 선취아원수치적IIM환자198례,기중유년형피기염(JDM)15례,DM 133례,PM 50례.기타CTD포괄SLE 70례,RA 60례,SSc 15례,pSS 46례,MCTD 10례화건강대조60명.채용ELISA법측정상술인군혈청중항NXP-2항체,항체양성혈청채용면역침정법진일보험증.채용t검험、Mann-Wittney U검험화x2검험등진행통계학분석.결과 혈청항NXP-2항체재IIM중적양성솔위5.1％(10/198),기중JDM조양성솔위20.0％(3/15),DM조3.7％(5/133),PM조4.0％(2/50),3조간차이유통계학의의(P＜0.05).이재기타CTD조급건강대조조적양성솔균위0.항NXP-2항체양성적IIM환자발병년령[(33±20)세]저우항체음성조[(45±17)세](t=-2.09,P＜0.05),피하개질침적적발생솔[30.0％(3/10)]고우항체음성조[2.6％(5/188)] (x2=0.7,P＜0.01),이2조간성별、병정、관절염、피진、근단기무력、기통、탄인곤난、합병간질성폐병화종류등적발생솔차이무통계학의의.수방분석,10례항체양성적IIM환자중,3례위JDM,기중1례JDM재수방10개월후사망,해환인혈청항NXP-2항체적도명현승고,합병엄범적피하개질침적,엄중적기무력화쾌속진전적병정.결론 본연구수차대아국IIM급기타CTD환자혈청중항NXP-2항체진행검측,발현항NXP-2항체시IIM적특이성항체,해항체양성자구유독특적림상표형.개전해항체적검사대IIM진단유중요개치,이차대IIM적림상분형유중요적지도의의.
Objective To determine the sera levels of anti-nuclear matrix protein (NXP)-2 autoantibodies and their clinical associations in patients with idiopathic inflammatory myopathies (IIM).Methods Sera from 198 Chinese patients with IIM including 15 juvenile dermatomyositis (JDM),133 dermatomyositis (DM) and 50 polymyositis (PM),other connective tissue diseases (CTDs) including 70 systemic lupus erythematosus,60 rheumatoid arthritis,15 systemic sclerosis,46 primary Sj(o)gren syndrome,10 mixed connective tissue disease and 60 healthy controls were measured by enzyme linked immunosorbent assay.The anti-NXP-2 antibodies were detected.The positive sera were further examined by immunoprecipitation assays.Statistical analyses were performed using student's t test or Mann-Wittney U test and x2 test.Results The positive rate of sera anti-NXP-2 autoantibodies in patients with IIM was 5.1％ (10/198),20.0％ (3/15) in patients with JDM,3.7％ (5/133) in patients with dermatomyositis,and 4.0％(2/50) in patients with polym-yositis.There was statistical significant difference in anti-NXP-2-positive rates between JDM,DM and PM (P＜0.05).However,the autoantibody did not present in patients with other CTDs as well as healthy controls.The anti-NXP-2-positive patients had significantly younger age [(33±20) vs (45±17) years old (t=-2.09,P＜0.05)] and higher incidence of calcinosis [30.0％(3/10) vs 2.6％(15/188)] compared with the anti-NXP-2-negativepatients (x2=0.7,P＜0.01).There were no statistical difference between the two groups in gender,disease duration,arthritis,rash,dysphagia,myasthenia,conccurrence with interstitial lung disease and cancer.In follow-up assessment,among the three JDM patients with anti-NXP-2 autoantibodies,one of them who died 10 months later had increased serum level of anti-NXP-2 autoantibody,extensive subcutaneous calcinosis,severe myasthenia and rapid progress.Conclusion This is the first report of the serum levels of anti-NXP-2 antibodies in Chinese patients with IIM and other CTDs.We find that anti-NXP-2 antibodies only exist in patients with IIM and are associated with early and calcinosis.