中华风湿病学杂志
中華風濕病學雜誌
중화풍습병학잡지
Chinese Journal of Rheumatology
2015年
9期
592-597
,共6页
皮肌炎%肺疾病,间质性%纵隔气肿
皮肌炎%肺疾病,間質性%縱隔氣腫
피기염%폐질병,간질성%종격기종
Dermatomyositis%Lung diseases,interstitial%Mediastinal emphysema
目的 探讨DM相关间质性肺疾病(ILD)合并纵隔气肿患者的临床特点及血清学特征.方法 收集2010年3月至2012年12月在我科住院的145例PM/DM患者的血清、临床资料和实验室数据.分别采用核酸免疫沉淀法和ELISA法检测抗氨基酰tRNA合成酶抗体和抗黑色素瘤分化相关基因蛋白(MDA)5抗体;采用免疫沉淀-蛋白印迹法榆测抗核基质蛋报2(NXP2)抗体、抗转录中介因子(TIF)1γ抗体、抗信号识别颗粒(SRP)抗体、抗小泛素样修饰物活化酶(SAE)抗体、抗Mi2抗体.按DM合并与未合并纵隔气肿,以及DM-ILD合并或未合并纵隔气肿进行分组,采用x2检验、Fisher精确概率法、t检验、Mann-Whitney U检验、Logistic多因素回归模型等进行统计学处理.结果 DM患者中共有11例合并自发性纵隔气肿,其中抗MDA5阳性10例,抗Mi2抗体阳性1例;无PM患者出现纵隔气肿.DM合并纵隔气肿组与未合并纵隔气肿组相比,合并纵隔气肿组急性进展犁ILD(RP-ILD)发生率(63.6％与24.4％,x2=7.25,P=0.01)、抗MDA5抗体阳性率(90.9％与52.4％,x2=5.86,P=0.02)、临床无肌病性皮肌炎(CADM)亚型比例(63.6％与22.0％,x2=8.57,P=0.007)、皮肤溃疡发生率(36.4％与11％,x2=5.20,P=0.04)更高,但CK水平较低[58.5(30.5,394.3) U/L与284(73.0,917.0) U/L,t=207.5,P=0.04].Logistic回归分析显示仅有皮肤溃疡是纵隔气肿发生的独立危险因素[OR=5.98,95％可信区间(95％CI)(1.12,31.98),P=0.037].在DM-ILD合并与未合并纵隔气肿组的比较中,合并纵隔气肿组CADM亚型比例更高(63.6％与27.9％,x2=5.37,P=0.03),但RP-ILD发生率、抗MDA5抗体阳性率、皮肤溃疡发生率和CK水平的差异无统计学意义.所有合并纵隔气肿患者均接受了激素和免疫抑制剂治疗,随访过程中6例死于呼吸衰竭.结论 纵隔气肿是DM的一种难治性并发症,预后较差;患者多属于CADM亚型、CK水平较低、合并RP-ILD、抗MDA5抗体阳性且容易出现皮肤溃疡.
目的 探討DM相關間質性肺疾病(ILD)閤併縱隔氣腫患者的臨床特點及血清學特徵.方法 收集2010年3月至2012年12月在我科住院的145例PM/DM患者的血清、臨床資料和實驗室數據.分彆採用覈痠免疫沉澱法和ELISA法檢測抗氨基酰tRNA閤成酶抗體和抗黑色素瘤分化相關基因蛋白(MDA)5抗體;採用免疫沉澱-蛋白印跡法榆測抗覈基質蛋報2(NXP2)抗體、抗轉錄中介因子(TIF)1γ抗體、抗信號識彆顆粒(SRP)抗體、抗小汎素樣脩飾物活化酶(SAE)抗體、抗Mi2抗體.按DM閤併與未閤併縱隔氣腫,以及DM-ILD閤併或未閤併縱隔氣腫進行分組,採用x2檢驗、Fisher精確概率法、t檢驗、Mann-Whitney U檢驗、Logistic多因素迴歸模型等進行統計學處理.結果 DM患者中共有11例閤併自髮性縱隔氣腫,其中抗MDA5暘性10例,抗Mi2抗體暘性1例;無PM患者齣現縱隔氣腫.DM閤併縱隔氣腫組與未閤併縱隔氣腫組相比,閤併縱隔氣腫組急性進展犛ILD(RP-ILD)髮生率(63.6％與24.4％,x2=7.25,P=0.01)、抗MDA5抗體暘性率(90.9％與52.4％,x2=5.86,P=0.02)、臨床無肌病性皮肌炎(CADM)亞型比例(63.6％與22.0％,x2=8.57,P=0.007)、皮膚潰瘍髮生率(36.4％與11％,x2=5.20,P=0.04)更高,但CK水平較低[58.5(30.5,394.3) U/L與284(73.0,917.0) U/L,t=207.5,P=0.04].Logistic迴歸分析顯示僅有皮膚潰瘍是縱隔氣腫髮生的獨立危險因素[OR=5.98,95％可信區間(95％CI)(1.12,31.98),P=0.037].在DM-ILD閤併與未閤併縱隔氣腫組的比較中,閤併縱隔氣腫組CADM亞型比例更高(63.6％與27.9％,x2=5.37,P=0.03),但RP-ILD髮生率、抗MDA5抗體暘性率、皮膚潰瘍髮生率和CK水平的差異無統計學意義.所有閤併縱隔氣腫患者均接受瞭激素和免疫抑製劑治療,隨訪過程中6例死于呼吸衰竭.結論 縱隔氣腫是DM的一種難治性併髮癥,預後較差;患者多屬于CADM亞型、CK水平較低、閤併RP-ILD、抗MDA5抗體暘性且容易齣現皮膚潰瘍.
목적 탐토DM상관간질성폐질병(ILD)합병종격기종환자적림상특점급혈청학특정.방법 수집2010년3월지2012년12월재아과주원적145례PM/DM환자적혈청、림상자료화실험실수거.분별채용핵산면역침정법화ELISA법검측항안기선tRNA합성매항체화항흑색소류분화상관기인단백(MDA)5항체;채용면역침정-단백인적법유측항핵기질단보2(NXP2)항체、항전록중개인자(TIF)1γ항체、항신호식별과립(SRP)항체、항소범소양수식물활화매(SAE)항체、항Mi2항체.안DM합병여미합병종격기종,이급DM-ILD합병혹미합병종격기종진행분조,채용x2검험、Fisher정학개솔법、t검험、Mann-Whitney U검험、Logistic다인소회귀모형등진행통계학처리.결과 DM환자중공유11례합병자발성종격기종,기중항MDA5양성10례,항Mi2항체양성1례;무PM환자출현종격기종.DM합병종격기종조여미합병종격기종조상비,합병종격기종조급성진전리ILD(RP-ILD)발생솔(63.6％여24.4％,x2=7.25,P=0.01)、항MDA5항체양성솔(90.9％여52.4％,x2=5.86,P=0.02)、림상무기병성피기염(CADM)아형비례(63.6％여22.0％,x2=8.57,P=0.007)、피부궤양발생솔(36.4％여11％,x2=5.20,P=0.04)경고,단CK수평교저[58.5(30.5,394.3) U/L여284(73.0,917.0) U/L,t=207.5,P=0.04].Logistic회귀분석현시부유피부궤양시종격기종발생적독립위험인소[OR=5.98,95％가신구간(95％CI)(1.12,31.98),P=0.037].재DM-ILD합병여미합병종격기종조적비교중,합병종격기종조CADM아형비례경고(63.6％여27.9％,x2=5.37,P=0.03),단RP-ILD발생솔、항MDA5항체양성솔、피부궤양발생솔화CK수평적차이무통계학의의.소유합병종격기종환자균접수료격소화면역억제제치료,수방과정중6례사우호흡쇠갈.결론 종격기종시DM적일충난치성병발증,예후교차;환자다속우CADM아형、CK수평교저、합병RP-ILD、항MDA5항체양성차용역출현피부궤양.
Objective To explore the clinical and serological features of patients with pneumomdiastinum (PNM) and dermatomyositis associated interstitial lung disease (DM-ILD).Methods A total of 145 polymyositis/dermatomyositis (PM/DM) patients hospitalized in our department from March,2010 to December,2012 were recruited.The sera,clinical and laboratory data were collected.Anti-aminoacyl-tRNA synthetase (anti-ARS) and anti-melanoma differentiation-associated gene 5 (MDA5) antibodies were detected by RNA-immunoprecipitation (RNA-IP) and ELISA,respectively.Anti-NXP2,anti-TIF1γ,anti-SRP,anti-SAE and antiMi2 antibodies were detected by immunoprecipitation-Western blotting.Variables were compared between DM patients with and without PNM,as well as between DM-ILD patients with and without PNM.Chi square test,Fisher's exact test,t test,Mann-Whitney U test and a multivariate logistic regression model were used for statistical analysis.Results A total of 11 DM patients (10 patients positive for anti-MDA5 and 1 patient positive for anti-Mi2) developed spontaneous PNM.No PM patient developed PNM.No differences of sex,age at the onset of DM,serum ferritin levels and C reactive protein (CRP) levels was observed between DM patients with and without PNM.Compared with DM patients without PNM,DM patients with PNM had significantly higher frequencies of rapidly progressive ILD (RP-ILD) (63.6％ vs 24.4％,x2=7.25,P=0.01),anti-MDA5 antibodies (90.9％ vs 52.4％,x2=5.86,P=0.02),clinically amyopathic DM (CADM) (63.6％ vs 22.0％,x2=8.57,P=0.007) and cutaneous ulcers (36.4％ vs 11％,x2=5.20,P=0.04),but significantly lower creatine kinase (CK) levels [58.5 (30.5,394.3) U/L vs 284 (73.0,917.0) U/L,t=207.5,P=0.04].The logistic multivariate analysis indicated that cutaneous ulcer was the only independent risk factor for the occurrence of PNM in DM [OR=5.98,95％ confidence interval (CI) (1.12,31.98),P=0.037].In the comparisons between DM-ILD patients with and without PNM,a higher frequency of CADM was observed in the PNM group (63.6％ vs 27.9％,x2=5.37,P=0.03),but no significant difference of the frequencies of RP-ILD,anti-MDA5 antibodies,cutaneous ulcers and CK levels was found in these two groups.All patients with PNM were treated with corticosteroids and immunosuppressants.During the follow-up period,6 patients died of respiratory failure.Conclusion Spontaneous PNM is a refractory complication with poor prognosis,and tends to occur in DM patients with RP-ILD,anti-MDA5 antibody,CADM diagnosis and low CK level,especially in patients with cutaneous ulcers.
