血清抗氨酰tRNA合成酶抗体检测在特发性炎性肌病伴间质性肺疾病中的意义

目的 检测多发性肌炎/皮肌炎患者血清抗氨酰tRNA合成酶(ARS)抗体水平,并与抗Jo-1抗体相比较,探讨其在特发性炎性肌病伴间质性肺疾病(ILD)中的意义.方法 采用酶联免疫吸附试验(ELISA)法测定109例多发性肌炎/皮肌炎患者,20例系统性红斑狼疮(SLE)患者,20例类风湿关节炎(RA)患者以及30名健康对照组血清中抗ARS抗体水平及阳性率.使用t检验、Mann-Wittney U检验、X2检验及Fisher精确检验分析ARS阳性患者的临床特点.使用McNemar检验比较抗ARS抗体和抗Jo-1抗体对诊断多发性肌炎/皮肌炎合并ILD的敏感性及特异性.结果 血清抗ARS抗体的阳性率在合并ILD的多发性肌炎/皮肌炎组、未合并ILD的多发性肌炎/皮肌炎组、SLE组、RA组及健康对照组分别是37.9％、7.8％、10％、0和0.合并ILD的多发性肌炎/皮肌炎组血清抗ARS抗体阳性率较未合并ILD的多发性肌炎/皮肌炎组、SLE组、RA组和健康对照组均显著升高(X2-13.5,5.45,10.57,15.17;P＜0.01).抗ARS抗体诊断多发性肌炎/皮肌炎合并ILD的敏感性显著高于抗Jo-1抗体(37.9％比17.2％,P＜0.01),而两者特异性差异无统计学意义(P＞0.05).抗ARS抗体阳性患者发热、ILD发生率较抗ARS抗体阴性患者显著升高(X2=12.55,13.53;均P＜0.01),而披肩征及向阳疹的发生率则低于ARS阴性组(X2=5.7,5.8;P均＜0.05).抗ARS抗体阳性组同抗Jo-1抗体阳性组间临床特点差异无统计学意义(P均＞0.05).随访发现多发性肌炎/皮肌炎合并ILD死亡患者血清抗ARS抗体均为阴性.结论 相较抗Jo-1抗体而言,血清抗ARS抗体检测对多发性肌炎/皮肌炎伴ILD的诊断敏感性更高,有利于早期诊断,值得临床推广应用.     

血清CEA和CA125水平对间质性肺病癌变的早期诊断学研究

目的比较ILD患者和ILD合并肺癌患者血清CEA和CA125的水平,探讨其在ILD合并肺癌早期诊断中的临床应用价值。方法设置ILD合并肺癌组(n=42)、ILD组(n=70)和对照组(n=60),测定并比较两组患者血清CEA和CA125的水平,通过受试者工作曲线评价CEA和CA125对ILD合并肺癌的诊断价值。结果 ILD合并肺癌组血清CEA、CA125水平均高于ILD组和对照组(P0.05);ILD组血清CEA、CA125水平均高于对照组(P0.05);血清CEA和CA125在诊断ILD合并肺癌的曲线下面积(area under curve,AUC)分别为0.722(95%CI:0.633~0.769,P0.05),0.751(95%CI:0.658~0.791,P0.05)。当血清CEA为6.2 ng/m L时,其诊断的敏感度为72.3%,特异度为69.2%;当血清CA125为60.4U/m L时,其诊断的敏感度为75.3%,特异度为79.7%。结论 ILD合并肺癌患者血清CEA和CA125较ILD患者要高,血清CEA和CA125对ILD合并肺癌的早期诊断具有参考价值。     

结缔组织病合并间质性肺病重症感染预后因素分析

目的回顾分析结缔组织病(CTD)合并间质性肺病(ILD)重症感染的危险因素、死亡原因及治疗方法。方法回顾性分析2014年5月至2017年5月RICU收治的67例CTD合并ILD终末期患者,明确诊断为CTD,包括系统性红斑狼疮(SLE)、类风湿关节炎(RA)、多发性肌炎/皮肌炎(PM/DM)、系统性硬化病(SSC)、干燥综合征(p SS)等,分析ILD发生情况及临床资料、治疗及预后情况。结果 1.存活组与死亡组患者在临床症状、体征、合并基础病、影像学组间差异无统计学意义。临床症状两组均有发热、咳嗽、呼吸困难为主要表现,影像以网格、磨玻璃、蜂窝及实变为主要表现。2.两组实验室检查比较,死亡组患者Ig G、Ig A、Ig M水平明显高于存活组(P 0. 05)、死亡组患者氧合指数明显低于存活组(P 0. 05)。3.双因素logistic回归,结果示有创呼吸机通气和无创呼吸机通气是影响预后的独立预测因子。4.本实验中将积极治疗后入ICU,是否行无创呼吸机通气和有创呼吸机各分为两组,分别统计患者的生存期,以28天生存期为上界,生存曲线显示,应用有创呼吸机组患者的生存期为13. 1±1. 73天,未应用有创呼吸机患者的生存期为19. 1±2. 22天,两者具有显著差别(P=0. 003)。在无创呼吸机组,生存期无统计学意义(P 0. 05)。结论 CTD合并ILD死亡组患者中Ig G、Ig A和Ig M显著升高,氧合指数明显降低,有创呼吸机通气和无创呼吸机通气是影响预后的独立预测因子,应用有创呼吸机患者的生存期明显低于未应用有创呼吸机组。建议患者CTD合并ILD终末期患者首先给予经鼻高流量吸氧,并尽量避免行有创机械通气和无创通气拖延抢救时机。     

血清KL-6检测在特发性炎性肌病伴肺间质病变诊断中的价值

目的探讨血清KL-6检测在特发性炎性肌病(IIM)合并肺间质病变(ILD)诊断中的作用。方法多发性肌炎(PM)和皮肌炎(DM)患者53例,其中合并有ILD的22例,正常对照者50名及肺部感染患者22例。酶联免疫吸附试验(ELISA)检测血清KL-6浓度。分析PM/DM患者临床表现及预后与血清KL-6水平的相关性。结果血清KL-6浓度在合并ILD的PM/DM组、未合并ILD的PM/DM组、肺部感染组和正常对照组的平均值分别是(1543±761)、(429±106)、(336±196)和(289±105)U/ml。合并ILD的PM/DM组血清KL-6水平较未合并ILD的PM/DM组、肺部感染组和正常对照组均显著升高(P＜0．01)。而未合并ILD的PM/DM组、肺部感染组和正常对照组间差异无统计学意义(P＞0．05)。血清KL-6的升高与肺间质病变呈显著正相关(P＜0．01),KL-6诊断PM/DM合并ILD的敏感性为90．9%,特异性为80．6%。随访分析表明,6例死亡的PM/DM患者血清KL-6水平明显高于其他伴发ILD的PM/DM患者(P＜0．01)。结论血清KL-6浓度是PM/DM合并ILD特异和敏感的血清学指标,它可用来鉴别肺部感染和肺间质病变。高水平的血清KL-6浓度可能提示预后差。     

临床无肌病性皮肌炎并间质性肺病分析

目的 提高对临床无肌病性皮肌炎(CADM)的认识,了解CADM合并肺间质病变(ILD)的临床特点.方法 回顾性研究109例DM患者症状、体征、实验室检查、胸X线片、HRCT、肺功能测定及动脉血气分析等.结果 109例皮肌炎(DM)中有CADM 17例(15.6%),女:男为4.7:1;发病年龄2～65岁,平均(43±14)岁:病程24～108个月,平均(29±27)个月;合并ILD者9例,未合并ILD者8例.CADM-ILD中,9例HRCT均表现网状斑片影;肺功能检查8例异常,其中限制性通气功能障碍6例,弥散功能障碍5例;6例存在不同程度的低氧血症,均无二氧化碳潴留;UCG检查测定轻至中度肺动脉高压4例;5例行肺活检示间质炎症,纤维化.17例患者全部给予激素,12例加用免疫抑制剂,重症予MP、静脉内免疫球蛋白(IVIG)冲击治疗.2例合并肺部感染于1个月内呼吸衰竭死亡.结论 本组CADM中35.3%有肌肉受累的亚临床证据;发生肺间质病变率52.9%,未合并感染时多表现为慢性进展.     

血清抗黑色素瘤分化相关基因抗体检测在多发性肌炎/皮肌炎患者中的意义

目的 检测多发性肌炎/皮肌炎(PM/DM)患者血清抗黑色素瘤分化相关基因(MDA5)抗体水平,探讨其在多发性肌炎/皮肌炎患者中的意义.方法 采用酶联免疫吸附试验(ELISA)法测定113例多发性肌炎/皮肌炎、30例系统性红斑狼疮(SLE),30例类风湿关节炎(RA),15例原发性干燥综合征(pSS),21例急性肺部感染患者以及50名健康对照组血清中抗MDA5抗体的阳性率.统计学方法采用t检验、Mann-Wittney U检验、X2检验、Fisher检验及Logistic多因素回归分析.结果 血清抗MDA5抗体的阳性率在多发性肌炎组、皮肌炎组、SLE组、RA组、SS组、肺部感染组及健康对照组分别是0、22.6％、3.3％、3.3％、0、0和0.皮肌炎组血清抗MDA5抗体阳性率(22.6％)明显高于多发性肌炎患者(0,P＜0.01),亦高于SLE患者(3.3％,X2=5.68,P＜0.05)、RA患者(3.3％,X2=5.68,P＜0.05)、pSS患者(0,P＜0.05)、肺部感染患者(0,P＜0.05)及健康对照(0,P＜0.01).皮肌炎亚组中,临床无肌病性皮肌炎(CADM)患者抗MDA5抗体阳性率(62.5％)较经典皮肌炎( CDM)患者高(18.4％,P＜0.05).抗MDA5抗体阳性的皮肌炎患者关节炎、发热、Ⅴ字疹、癌胚抗原升高、CA153升高的发生率及血清谷氨酰转肽酶(GGT)、铁蛋白浓度较抗MDA5抗体阴性患者显著升高(X2=4.08,8.06,6.357,32.4,4.867;Z=-2.86,-2.44;P均＜0.05);而抗核抗体阳性、血清肌酸激酶浓度及外周血T细胞、自然杀伤细胞计数则低于抗MDA5阴性组(X2=4.08;Z=-2.072,-2.013,-2.907;P均＜0.05).此外,抗MDA5抗体阳性的皮肌炎患者急性/亚急性间质性肺炎(A/SIP)的发生率(78.9％)显著高于阴性患者(3.2％)(P＜0.01).抗MDA5抗体诊断皮肌炎合并A/SIP的敏感性为88.2％,特异性为94％.多因素分析发现血清抗MDA5阳性为皮肌炎合并间质性肺疾病(ILD)患者死亡的独立危险因素(0R=8.46,95％CI 1.77～40.36,P＜0.01).结论 在我国多发性肌炎/皮肌炎患者人群,血清抗MDA5抗体主要存在于皮肌炎患者,是皮肌炎合并A/SIP的敏感及特异性血清学指标,也是皮肌炎合并ILD死亡的独立危险因素.     

多发性肌炎/皮肌炎合并肺间质病变的临床特征和预后

目的 研究多发性肌炎(PM)和皮肌炎(DM)合并肺间质病变(ILD)的临床特点和预后.方法 回顾性分析107例PM/DM患者的临床资料,包括首发症状、临床表现、实验室检查、影像学资料、治疗及预后.结果 107例PM/DM患者合并ILD有28例,ILD发生率为26.2%.①合并ILD的首发症状为关节炎或关节痛者高于无ILD(P<0.05);合并ILD临床表现为关节炎或关节痛、发热、干咳气促者明显高于无ILD(P<0.05).②DM合并ILD患者大多有特异性皮疹,呼吸困难较重(P<0.05),而PM合并ILD患者肌痛、肌无力较重(P<0.05).③合并ILD的红细胞沉降率(ESR)和C反应蛋白(CRP)明显高于无ILD(P<0.05);DM-ILD组肌酶谱以羟丁酸脱氢酶(HBDH)、天冬氨酸转氨酶(AST)升高为主(P≤0.05),PM合并ILD患者肌酶谱以肌酸激酶(CK)和肌酸激酶同工酶(CK-MB)为主(P<0.05).④合并ILD的28例PM/DM患者经治疗,20例病情改善,8例重症7例均为DM合并ILD,5例治疗无效因Ⅰ型呼吸衰竭死亡(病死率占PM/DM合并ILD患者的17.9%).结论 ①首发症状为关节炎或关节痛,临床表现有关节炎或者关节痛、发热以及ESR和CRP高者易合并ILD;有特异性皮疹、AST升高的DM易合并ILD;肌酶以CK和CK-MB升高为主的PM易合并ILD.②DM合并ILD病情进展凶险,病死率高,预后不良.     

原发性干燥综合征合并间质性肺病的临床特征

目的探讨原发性干燥综合征（primary Sjogen’s syndrome,pSS）合并间质性肺病（interstitial lung disease．ILD）的临床及影像学特点,以提高对原发性干燥综合征合并间质性肺病（pSS—ILD）的认识。方法回顾性分析本院2004年1月．2007年12月确诊且资料完整的219例pSS患者,对其中47例合并ILD患者的临床特点及影像学检查进行分析。结果（1）pSS组ILD的发生率为21．46％,pSS-ILD组发病年龄晚于pSS一无ILD组,且病程长于pSS．无ILD组（P〈O．01）;（2）pSS-ILD组血沉（erythroeyte sedimentation rate,ESR）明显增快,C反应蛋白（C-reaetive protein,CRP）明显增高,两者与pSS-无ILD组比较差异均具有统计学意义（P〈0．01）;（3）pSS-ILD组抗SSA抗体阳性率高于pSS-无ILD组（P〈0．05）;抗核抗体（antinuclear antibody,ANA）、类风湿因子（rheumatoid factor。RF）、抗SSB抗体、血浆球蛋白〉30g／L、低补体C3和低补体C4在两组间无明显差异;（4）在诊断pSS—ILD病变时,肺高分辨率CT（high resolution computerized tomography,HRCT）明显优于普通X线胸片。结论ILD是pSS常见的系统损害。pSS-ILD的发生与疾病的活动及某些自身抗体有关,肺HRCT有助于pSS—ILD患者的早期诊断及预后判断。     

类风湿关节炎合并间质性肺病患者血清中转化生长因子-β1骨形成蛋白-4检测及其意义

目的 探讨类风湿关节炎(RA)合并间质性肺病( ILD)患者的临床特点和转化生长因子(TGF)-β1、骨形成蛋白(BMP)-4在血清中的水平.方法 人选28例RA-ILD患者、32例单纯RA患者和20名健康对照,RA-ILD组进一步分为早期组(16例)和中晚期组(12例),酶联免疫吸附试验(ELISA)法测定血清中TGF-β1、BMP-4水平,并分析血清TGF-β1、BMP-4水平与实验室指标的相关性.采用t检验、单因素方差分析及Spearman相关分析法进行数据分析.结果 与单纯RA患者相比,RA-ILD组患者初次发生关节炎时年龄较大,关节功能优于单纯RA组患者,血清类风湿因子(RF)滴度高(P＜0.05,P＜0.05,P＜0.01),出现肺间质病变时间为2～6年,平均(3.0±1.2)年.TGF-β1水平在RA-ILD组稍增高,但3组相比差异无统计学意义(P＞0.05).早期RA-ILD组患者TGF-β1血清水平明显增高,与中晚期RA-ILD组及单纯RA组、健康对照组相比较差异均有统计学意义(P＜0.01).RA-ILD组患者BMP-4水平低于单纯RA组及健康对照组,差异有统计学意义(P＜0.05),且早期RA-ILD组患者BMP-4水平显著下降,与中晚期RA-ILD组及单纯RA组、健康对照组相比较差异均有统计学意义(P＜0.05).血清BMP-4水平与TGF-β1水平之间无相关性(P＞0.05),血清TGF-β1、BMP-4水平与实验室指标红细胞沉降率、C反应蛋白、RF、抗环瓜氨酸肽(CCP)抗体等均无明显相关性(P均＞0.05).结论 在早期RA-ILD患者中血清TGF-β1水平增高,BMP-4水平减低,二者可能参与了RA患者ILD的发生.     

血清表面活性蛋白与系统性红斑狼疮合并间质性肺病的相关性研究

目的 探讨血清表面活性蛋白A(SP-A)和D(SP-D)与系统性红斑狼疮(SLE)合并间质性肺病(ILD)的相关性及其临床意义.方法 采用双抗体夹心酶联免疫吸附试验(ELISA)检测,并比较SLE组和对照组血清样本SP-A和SP-D水平的差异,分析其与SLE合并ILD的关系,判断与肺部高分辨率CT(HRCT)评分、肺功能、年龄、病情活动指标之间的相关性.结果 SLE组患者血清SP-A和SP-D水平高于健康对照组(P＜0.05).SLE组并发ILD患者血清SP-A和SP-D水平高于未合并ILD者以及对照组(P＜0.05).合并ILD的SLE患者血清SP-D水平与HRCT磨玻璃影评分(r=0.508,P=0.004)和间质病变评分(r=0.468,P=0.009)呈正相关关系,与肺活量(%VC)(r=-0.590,P=0.001)和一氧化碳弥散量(%DLCO)(r=-0.588,P＜0.01)呈负相关关系,而SP-A与上述指标无明显相关.SLE患者血清SP-D水平与年龄呈正相关关系(r=0.352,P=0.001).SLE-ILD组血清SP-D水平与血清IsG(r=0.376,P=0.040)呈正相关关系,SP-A水平与C反应蛋白(CRP)(r=0.403,P=0.027)呈正相关关系.结论 SP-D和SP-A是SLE并发ILD的血清学标志,SP-D与患者的HRCT评分、肺功能指标、年龄和病情活动度相关.     

原发性干燥综合征90例血清C反应蛋白转化生长因子-β1水平变化及意义

目的:探讨血清CRP、TGF-β1与pSS的联系。方法:选择90例pSS患者(观察组)及30名健康人群(对照组),观察组按病情严重不同分为重度17例、中度43例、轻度30例,观察组中未发生肺间质改变(ILD)54例、合并ILD 36例(其中Ⅰ期患者15例、Ⅱ期患者13例、Ⅲ期患者8例)。ELISA测定患者和健康人血清中CRP、TGF-β1水平,并探讨其和pSS严重程度及ILD不同分期的联系。统计方法使用方差分析,SNK-q检验或t检验,Spearman等级相关分析等。结果:观察组血清CRP、TGF-β1水平分别为[(13.5±7.8)mg/L],[(61±14)μg/L],高于对照组[(3.5±1.1)mg/L]和[(28±9)μg/L],差异有统计学意义(t=6.980,P<0.01;t=12.086,P<0.01)。在观察组内,病情中、重度的患者其血清CRP浓度分别为[(14.6±2.1)mg/L]和[(16.5±3.3)mg/L],比轻度患者[(11.3±3.2)mg/L]高,病情中、重度的患者其血清TGF-β1浓度分别为[(61±13)μg/L]和[(76±18)μg/L],比轻度患者[(51±14)μg/L]高,而且重度患者的水平提高的更显著(F=3.634,P<0.01;F=4.661,P<0.01);血清CRP、TGF-β1浓度和病情严重度呈正相关[r=0.786,P<0.01;r=0.516,P<0.01]。观察组内患者合并有ILD的患者,其血清CRP、TGF-β1浓度分别为[(15.5±3.2)mg/L]和[(74±19)μg/L],比无合并ILD的患者[(10.5±2.2)mg/L]和[(52±14)μg/L)]高(t=8.791,P<0.01;t=6.321,P<0.01)。观察组内,ILDⅡ、Ⅲ期的患者,其血清CRP浓度分别为[(16.3±8.2)mg/L]和[(19.1±10.1)mg/L],比Ⅰ期患者[(13.4±7.3)mg/L]高,TGF-β1浓度分别为[(74±19)μg/L]和[(85±21)μg/L],比Ⅰ期患者[(63±15)μg/L]高,Ⅲ期的患者水平高的更显著(F=4.640,P<0.01;F=5.673,P<0.01);pSS患者血清CRP、TGF-β1的浓度和ILD的分期都呈正相关关系(r=0.718,P<0.01;r=0.809,P<0.01)。结论:pSS患者血清CRP、TGF-β1浓度都较健康人升高,这2种因子很可能和pSS病情活动以及合并的ILD的转归有联系。     

Clinical Heterogeneity of Interstitial Lung Disease in Polymyositis and Dermatomyositis Patients With or Without Specific Autoantibodies

Background

The aim of this study was to compare the heterogeneity of interstitial lung disease (ILD) in patients with polymyositis and dermatomyositis (PM/DM) according to serological type.

Tofacitinib treatment in anti-glycyl-tRNA synthetase antibody interstitial lung disease – A case report

Anti-aminoacyl-transfer-RNA synthetase syndrome (ASS) related interstitial lung disease (ILD) is rarely presented initially alongside acute respiratory distress syndrome (ARDS), which in and of itself is a severe condition with a high mortality rate. Additionally, rapidly progressive change is not a common feature in ASS. Numerous case reports have described the efficacy which tofacitinib has on rapidly progressive ILD (RP-ILD). However, none have mentioned the use of tofacitinib in patients with impaired renal function. Herein, a case of ASS involving ILD is reported with the initial presentation of RP-ILD to ARDS being complicated by acute renal failure with an initial complete response to tofacitinib. Patients experiencing unexplained rapidly progressive interstitial pneumonia should be examined thoroughly for the diagnosis of ASS. Furthermore, tofacitinib can also be considered as a choice of treatment even in patients with impaired renal function.     

抗Ro52抗体阳性的抗合成酶综合征患者的临床特征分析

分析抗Ro52抗体阳性的抗合成酶综合征（ASS）患者的临床特征。收集2017年至2020年郑州大学第一附属医院风湿免疫科和呼吸科住院确诊的203例ASS患者,回顾性分析其一般资料、临床表现、实验室检查等。203例ASS患者中男55例,女148例,ASS发病年龄（51.9±13.3）岁。抗Ro52抗体阳性者163例,抗R...     

Anti-MDA5 antibody dermatomyositis-associated rapidly progressive interstitial lung disease patient complicated with mixed connective tissue disease: A case report

Anti-MDA5 antibody dermatomyositis (DM) is a special type of myositis, which can potentially cause rapidly progressive interstitial lung disease (RP-ILD). Mixed connective tissue disease (MCTD) is a complex disease with different characteristics of autoimmune connective tissue disease, associated with ILD. Both are rare diseases, and few patients with both diseases have been reported. A 71-year-old woman complained of palpitations, with a 2 months history of rash around her hands, extensor surface of right elbow, and the nape of her neck. Subsequently, the patient had acute exacerbation of dyspnea and tachypnea. Anti-Ro52, U1 RNP and MDA5 antibodies were positive; the presenting evidence was suggestive of anti-MDA5⁺DM-RP-ILD complicated with MCTD. Our patient deteriorated rapidly and had a fatal outcome, despite “triple therapy” for RP-ILD. This case illustrates that patients with coexisting anti-MDA5⁺DM and MCTD have the former's typical clinical manifestations, and may develop ILD quickly rather than slowly as in MCTD, especially with the coexistence of anti-Ro52 antibodies.     

多发性肌炎/皮肌炎合并间质性肺疾病的相关因素及预后不良因素分析

目的 探讨多发性肌炎/皮肌炎(PM/DM)患者发生间质性肺疾病(ILD)的相关因素及影响预后的不良因素.方法 以上海第二军医大学长海医院1997年1月至2006年11月收住的PM/DM患者87例为研究对象,分为ILD组40例(男13例,女27例),平均年龄(54±13)岁;非ILD组47例(男25例,女22例),平均年龄(45±18)岁.对ILD的发生率、临床特征和预后进行分析.正态分布的计量资料采用t检验,偏态分布的计量资料采用秩和检验,计数资料两组率的比较采用x2检验,PM/DM伴发ILD的预测因素和预后不良因素采用logistic回归分析和Kaplan-Meier生存曲线.结果 PM/DM中ILD的发生率为46％(40/87),病死率为40％(16/40).ILD组的平均年龄[(54±13)岁]明显大于非ILD组[(45±18)岁];ILD组出现发热(21/40)、吞咽困难(16/40)、关节痛(26/40)、Gottron皮疹(14/40)和心脏损害(26/40)的百分率明显高于非ILD组(分别为7/47、8/47、9/47、2/47和14/47);ILD组的血清乳酸脱氢酶[(472±285)IU]和ESR[(44 ±24)mm/1 h]明显高于非ILD)组[(310±238)IU和(26±24)mm/1 h];ILD组的IgG[(18±9)g/L]明显高于非ILD组[(14±5)g/L].经多因素非条件logistic回归分析,筛选出4个与ILD相关的预测因子:Gottron皮疹、关节痛、发热和年龄≥40岁,其相对危险度分别为12.048、7.812、6.329和5.236;生存分析结果显示,Gottron皮疹、心脏损害和肺间质病变是影响ILD预后的不良因素.结论 PM/DM患者年龄≥40岁,出现Gottron皮疹、关节痛和发热与ILD的发生密切相关,Gottron皮疹、心脏损害和肺间质病变是影响ILD预后的不良因素.     

Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis

OBJECTIVE: To assess the difference in clinical features and prognosis of patients with interstitial lung disease (ILD) comparing polymyositis (PM) and dermatomyositis (DM). METHODS: Medical records of 28 ILD patients with PM/DM (16 PM-ILD, 12 DM-ILD) were reviewed retrospectively. RESULTS: Serum CPK concentrations were significantly higher in PM-ILD than in DM-ILD. Bronchoalveolar lavage analysis showed that the percentages of lymphocytes and eosinophils were significantly higher in DM-ILD than in PM-ILD. Ten patients (5 PM-ILD, 5 DM-ILD) underwent surgical lung biopsy, and 3 (3 DM-ILD) had an autopsy. Nonspecific interstitial pneumonia (NSIP) was found in 7 (4 PM-ILD, 3 DM-ILD) and usual interstitial pneumonia (UIP) in 3 (1 PM-ILD, 2 DM-ILD). Interestingly, diffuse alveolar damage (DAD) was found in 3 patients with DM-ILD, who all died of deterioration of ILD; but no one with PM-ILD had DAD. Corticosteroid treatment alone achieved a favorable response in 6 patients (37.5%) with PM-ILD, but in only one (8.3%) with DM-ILD. Administration of cyclosporine in the early phase of onset benefited 4 corticosteroid-resistant patients with DM-ILD. Conclusively, survival in DM-ILD was significantly worse than that in PM-ILD. CONCLUSION: DM-ILD is more refractory to corticosteroid therapy, resulting in poorer prognosis compared with PM-ILD. These data indicate that intensive therapy, including cyclosporine, should be considered for DM-ILD.     

Low positive titer of anti-melanoma differentiation-associated gene 5 antibody is not associated with a poor long-term outcome of interstitial lung disease in patients with dermatomyositis

Background

Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab) is associated with fatal rapidly progressive interstitial lung disease (RP-ILD) in patients with dermatomyositis (DM). We attempted to clarify whether anti-MDA5-Ab is associated with long-term outcomes in patients with DM-ILD.

KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis

Abstract. Fathi M, Barbasso Helmers S, Lundberg IE (Karolinska University Hospital, Stockholm; Karolinska Institutet, Karolinska University Hospital, Stockholm; and Institute of Environmental Medicine, Karolinska Institutet, Stockholm; Sweden). KL‐6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis. J Intern Med 2012; 271 : 589–597. Objectives. To investigate whether Caucasian patients with polymyositis (PM) or dermatomyositis (DM) and interstitial lung disease (ILD) have elevated serum levels of KL‐6 compared with patients without ILD and whether KL‐6 could be used as a marker for ILD activity and treatment efficacy of ILD in PM/DM. Design and methods. Thirty patients with PM/DM (seven with ILD) and 17 age‐ and sex‐matched healthy controls were included in a retrospective, cross‐sectional analysis. Twelve patients were followed for longitudinal evaluation. ILD was defined as restrictive lung function impairment with radiographic signs of ILD. Serum KL‐6 levels were measured using a sandwich enzyme immunoassay kit. Groups were compared by Mann–Whitney U‐test. Results. PM/DM patients with ILD had significantly higher median serum KL‐6 levels compared with those without ILD: 995 (range 533–2318) versus 322 (range 132–1225) U mL^?1 (P = 0.0002). Median serum levels of healthy controls were 225 (range 136–519) U mL^?1. Serum levels of KL‐6 were inversely correlated with percentages of forced expiratory volume in 1 s (FEV₁), vital capacity (VC), total lung capacity (TLC), forced VC, diffusing capacity of carbon monoxide (DLco), maximal voluntary ventilation at 40 breaths min^?1 and residual volume (RV). Changes in KL‐6 levels showed a significant inverse correlation with changes in percentage FEV₁, TLC, DLco and RV. At a cut‐off level of 549 U mL^?1 (mean ± 2.5 SD for controls), the sensitivity and specificity for diagnosis of ILD were 83% and 100%, respectively. Conclusion. The level of serum KL‐6 may serve as measure of ILD in patients with PM/DM and is a promising biomarker for use in clinical practice to assess clinical response to treatment.