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Article(id=1211268934809031655, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1211268928383348982, articleNumber=null, orderNo=null, doi=10.11855/j.issn.0577-7402.2021.02.06, pmid=null, cstr=null, oa=null, hot=null, price=null, onlineType=0, articleFormat=0, articleType=null, articleTypeStr=research-article, receivedDate=1584720000000, receivedDateStr=2020-03-21, revisedDate=1595779200000, revisedDateStr=2020-07-27, acceptedDate=null, acceptedDateStr=null, onlineDate=1766718618347, onlineDateStr=2025-12-26, pubDate=1614441600000, pubDateStr=2021-02-28, doiRegisterDate=null, doiRegisterDateStr=null, onlineIssueDate=1766718618347, onlineIssueDateStr=2025-12-26, onlineJustAcceptDate=null, onlineJustAcceptDateStr=null, onlineFirstDate=null, onlineFirstDateStr=null, sourceXml=null, magXml=null, createTime=1766718618347, creator=13701087609, updateTime=1766718618347, updator=13701087609, issue=Issue{id=1211268928383348982, tenantId=1146029695717560320, journalId=1189873630562394117, year='2021', volume='46', issue='2', pageStart='107', pageEnd='211', issueExtLink='null', onlineDate='null', pubDate='null', beforeIssueId=null, nextIssueId=null, price=null, status=1, issueComplete=1, articleOrder=1, issueType=-1, specialIssue=0, createTime=1766718616815, creator=13701087609, updateTime=1766718805938, updator=13701087609, preIssue=null, nextIssue=null, ext={EN=IssueExt(id=1211269721685627740, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1211268928383348982, language=EN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=), CN=IssueExt(id=1211269721685627741, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1211268928383348982, language=CN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=)}, issueFiles=null}, startPage=142, endPage=148, ext={EN=ArticleExt(id=1211268935140381687, articleId=1211268934809031655, tenantId=1146029695717560320, journalId=1189873630562394117, language=EN, title=Clinical characteristics analysis of 63 interstitial lung disease patients with positive myositis autoantibodies, columnId=1190310109000602400, journalTitle=Medical Journal of Chinese People’s Liberation Army, columnName=Clinical Research, runingTitle=null, highlight=null, articleAbstract=

Objective To analyze the clinical characteristics of myositis autoantibody detection in the diagnosis of interstitial lung disease (ILD), and explore the diagnosis and treatment of ILD with the help of clinical manifestations combined with antibody results. Methods Sixty-three patients of ILD accurately diagnosed with the help of myositis autoantibody detection were collected retrospectively and divided into three groups: anti-synthetase syndrome associated interstitial lung disease group(ASS-ILD group, n=22), polymyositis/dermatomyositis associated interstitial lung disease group (PM/DM-ILD group, n=18) and interstitial pneumonia with autoimmune features group (IPAF group, n=23). Their clinical characteristics, treatment and prognosis were analyzed and compared. Results The positive rates of Raynaud's phenomenon, joint symptoms, mechanic's hand and anti-ARS antibodies in ASS-ILD group were higher than those in IPAF group (P<0.0167). Compared with PM/DM-ILD group, ASS-ILD group had higher positive rates of Raynaud's phenomenon, cough, dyspnea, respiratory system symptoms as the first manifestation, anti-ARS antibodies but lower positive rates of fever, muscle symptoms, skin/muscle symptoms as the first manifestation (P<0.0167).Compared with IPAF group, PM/DM-ILD group had higher positive rates of muscle symptoms, skin symptoms, skin/muscle symptoms as the first manifestation, anti-ARS antibodies and anti-MDA5 antibody but lower positivity rates of respiratory system symptoms as the first manifestation (P<0.0167). In this study, chest radiological findings of all patients were mainly nonspecific interstitial pneumonia (NSIP). The abnormality in pulmonary functions manifested mainly as diffusion disturbance and restrictive ventilatory dysfunction. 87.3% of all patients were treated with glucocorticoid combined with immunosuppressants. After treatment, 81.0% of these patients showed improvement or stability, while 19.0% showed deterioration or death. The difference in prognosis between the three groups was not statistically significant (P>0.05). Conclusion The detection of myositis autoantibody should be paid more attention in ILD patients. Diagnosis, classification, treatment and prognosis evaluation of ILD could be made with the help of myositis autoantibodies.

, correspAuthors=Xiao-Kui Tang, authorNote=null, correspAuthorsNote=
*E-mail:
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目的 总结分析肌炎自身抗体阳性间质性肺疾病(ILD)的临床特点，评估肌炎自身抗体检测在ILD临床诊断中的价值。方法 回顾性分析重庆医科大学附属第一医院2018年1月—2019年9月收治的63例结合肌炎自身抗体明确诊断ILD患者的病例资料，分为抗合成酶综合征相关ILD(ASS-ILD)组(n=22)、多发性肌炎/皮肌炎相关ILD(PM/DM-ILD)组(n=18)与自身免疫特征的间质性肺炎(IPAF)组(n=23)，分析对比三组患者的临床特点、治疗及预后情况。结果 ASS-ILD组的雷诺现象、关节症状、技工手、抗ARS抗体阳性率高于IPAF组(P<0.0167)。ASS-ILD组的雷诺现象、咳嗽、呼吸困难、首发表现(呼吸系统症状)、抗ARS抗体阳性率高于PM/DM-ILD组，而发热、肌肉症状、首发表现(皮肤/肌肉症状)阳性率低于PM/DM-ILD组(P<0.0167)。PM/DM-ILD组的肌肉症状、皮肤症状、首发表现(皮肤/肌肉症状)、抗ARS抗体、抗MDA5抗体阳性率高于IPAF组，而首发表现(呼吸系统症状)阳性率低于IPAF组(P<0.0167)。63例患者胸部影像学特征以非特异性间质性肺炎(NSIP)表现为主，肺功能检查结果以弥散障碍及限制性通气障碍为主。87.3%的患者使用激素联合免疫抑制剂治疗。81.0%的患者转归为好转或稳定，19.0%的患者转归为进展恶化或死亡，三组患者转归差异无统计学意义(P>0.05)。结论 ILD患者须重视肌炎自身抗体的筛查，其结果可辅助用于ILD的诊断、分类及预后评估。

, correspAuthors=唐小葵, authorNote=null, correspAuthorsNote=
唐小葵，E-mail：
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周倩，硕士研究生，主要从事间质性肺疾病的临床研究。E-mail：

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Chin J Tuberc Respir Dis, 2019, 42(10): 765-770., articleTitle=Clinical value of myositis antibodies in patients with connective tissue disease-associated interstitial lung diseases, refAbstract=null), Reference(id=1211268948830589436, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, doi=null, pmid=null, pmcid=null, year=2019, volume=42, issue=10, pageStart=765, pageEnd=770, url=null, language=null, rfNumber=[22], rfOrder=27, authorNames=谢苗苗, 邹如意, 李燕, journalName=中华结核和呼吸杂志, refType=null, unstructuredReference=[谢苗苗, 邹如意, 李燕, 等. 结缔组织病相关间质性肺病患者肌炎抗体检测的临床意义[J]. 中华结核和呼吸杂志, 2019, 42(10): 765-770.], articleTitle=结缔组织病相关间质性肺病患者肌炎抗体检测的临床意义, refAbstract=null), Reference(id=1211268948914475518, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, doi=null, pmid=null, pmcid=null, year=2015, volume=10, issue=3, pageStart=e120313, pageEnd=null, url=null, language=null, rfNumber=[23], rfOrder=28, authorNames=Hozumi H, Enomoto N, Kono M, journalName=PLoS One, refType=null, unstructuredReference=Hozumi H, Enomoto N, Kono M, et al. 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PLoS One, 2015, 10(3): e120313., articleTitle=Prognostic significance of anti-aminoacyl-tRNA synthetase antibodies in polymyositis/dermatomyositis-associated interstitial lung disease: a retrospective case control study, refAbstract=null)], funds=[Fund(id=1211268944942469521, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, awardId=2020MSXM033, language=EN, fundingSource=Project of Chongqing Science and Health Joint Medical Research(2020MSXM033), fundOrder=null, country=null), Fund(id=1211268945022161300, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, awardId=2020MSXM033, language=CN, fundingSource=重庆市科卫联合医学科研项目(2020MSXM033), fundOrder=null, country=null)], companyList=[AuthorCompany(id=1211268937451442267, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, xref=null, ext=[AuthorCompanyExt(id=1211268937459830876, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, companyId=1211268937451442267, language=EN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=Department of Respiratory and Critical Medicine, the First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China), AuthorCompanyExt(id=1211268937468219485, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, companyId=1211268937451442267, language=CN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=重庆医科大学附属第一医院呼吸与危重症医学科，重庆 400016)])], figs=[ArticleFig(id=1211268943440908607, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=EN, label=Fig.1, caption=Chest CT findings of ILD patients, figureFileSmall=dh2//5Okbpy6pQ6Qo8A+CA==, figureFileBig=dq/Nr3KD8ZIZgUcDPV6xLw==, tableContent=null), ArticleFig(id=1211268943516406083, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=CN, label=图1, caption=ILD患者的胸部CT表现

ASS. 抗合成酶综合征；ILD. 间质性肺疾病；NSIP. 非特异性间质性肺炎；IPAF. 自身免疫特征的间质性肺炎；UIP. 普通型间质性肺炎；OP. 机化性肺炎；PM. 多发性肌炎/皮肌炎；AIP. 急性间质性肺炎

, figureFileSmall=dh2//5Okbpy6pQ6Qo8A+CA==, figureFileBig=dq/Nr3KD8ZIZgUcDPV6xLw==, tableContent=null), ArticleFig(id=1211268943793230160, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=EN, label=Fig.2, caption=Comparison of chest CT findings in ILD patients before and after treatment, figureFileSmall=vAXeSHIKZLNnHIUODwOLJg==, figureFileBig=/D4Yu2OJ3gVKRX2ZPFXLEA==, tableContent=null), ArticleFig(id=1211268943868727638, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=CN, label=图2, caption=ILD患者治疗前后胸部CT对比

ASS. 抗合成酶综合征；ILD. 间质性肺疾病；DM. 皮肌炎；IPAF. 自身免疫特征的间质性肺炎

, figureFileSmall=vAXeSHIKZLNnHIUODwOLJg==, figureFileBig=/D4Yu2OJ3gVKRX2ZPFXLEA==, tableContent=null), ArticleFig(id=1211268943944225114, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=EN, label=Tab.1, caption=

Comparison of general data among ILD patients in three groups

, figureFileSmall=null, figureFileBig=null, tableContent=
指标ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)F/χ2P
性别[例(%)]
 男13(59.1)8(44.4)13(56.5)0.9500.660
 女9(40.9)10(55.6)10(43.5)
年龄[岁，$\bar{x}±s$]62.4±11.357.5±16.063.2±16.60.8450.435
病程[月，M(Q1，Q3)]2(1，12)3.5(1，15)4.0(1，24)1.0810.582
首发表现[例(%)]
 呼吸系统症状22(100.0)(2)8(44.4)(1)(3)22(95.7)(2)21.8730.000
 皮肤/肌肉症状0(2)10(55.6)(1)(3)1(4.3)(2)
发热[例(%)]6(27.3)(2)12(66.7)(1)7(30.4)7.7130.022
皮肤症状[例(%)]5(22.7)10(55.6)(3)3(13.0)(2)9.5080.009
肌肉症状[例(%)]3(13.6)(2)14(77.8)(1)(3)3(13.0)(2)24.6450.000
关节症状[例(%)]13(59.1)(3)5(27.8)5(21.7)(1)7.5960.023
其余CTD表现[例(%)]8(36.4)2(11.1)8(34.8)3.7780.177
雷诺现象[例(%)]14(63.6)(2)(3)2(11.1)(1)2(8.7)(1)20.3960.000
技工手[例(%)]12(54.5)(3)4(22.2)2(8.7)(1)12.0800.002
咳嗽[例(%)]22(100.0)(2)13(72.2)(1)19(82.6)7.0360.025
呼吸困难[例(%)]21(95.5)(2)11(61.1)(1)21(91.3)8.5400.010
呼吸衰竭[例(%)]6(27.3)6(33.3)6(26.1)0.2880.866
爆裂音[例(%)]14(63.6)6(33.3)15(65.2)5.0510.098
C3/C4下降[例(%)]a6(33.3)4(30.8)5(23.8)0.5600.795
红细胞沉降率增高[例(%)]a8(47.1)11(68.8)13(68.4)2.1630.354
C反应蛋白＞10 mg/L[例(%)]13(59.1)13(72.2)13(56.5)1.1690.557
肌酶谱增高[例(%)]a11(57.9)10(58.8)7(46.7)0.5850.746
), ArticleFig(id=1211268944049082719, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=CN, label=表1, caption=

三组ILD患者一般资料比较

, figureFileSmall=null, figureFileBig=null, tableContent=
指标ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)F/χ2P
性别[例(%)]
 男13(59.1)8(44.4)13(56.5)0.9500.660
 女9(40.9)10(55.6)10(43.5)
年龄[岁，$\bar{x}±s$]62.4±11.357.5±16.063.2±16.60.8450.435
病程[月，M(Q1，Q3)]2(1，12)3.5(1，15)4.0(1，24)1.0810.582
首发表现[例(%)]
 呼吸系统症状22(100.0)(2)8(44.4)(1)(3)22(95.7)(2)21.8730.000
 皮肤/肌肉症状0(2)10(55.6)(1)(3)1(4.3)(2)
发热[例(%)]6(27.3)(2)12(66.7)(1)7(30.4)7.7130.022
皮肤症状[例(%)]5(22.7)10(55.6)(3)3(13.0)(2)9.5080.009
肌肉症状[例(%)]3(13.6)(2)14(77.8)(1)(3)3(13.0)(2)24.6450.000
关节症状[例(%)]13(59.1)(3)5(27.8)5(21.7)(1)7.5960.023
其余CTD表现[例(%)]8(36.4)2(11.1)8(34.8)3.7780.177
雷诺现象[例(%)]14(63.6)(2)(3)2(11.1)(1)2(8.7)(1)20.3960.000
技工手[例(%)]12(54.5)(3)4(22.2)2(8.7)(1)12.0800.002
咳嗽[例(%)]22(100.0)(2)13(72.2)(1)19(82.6)7.0360.025
呼吸困难[例(%)]21(95.5)(2)11(61.1)(1)21(91.3)8.5400.010
呼吸衰竭[例(%)]6(27.3)6(33.3)6(26.1)0.2880.866
爆裂音[例(%)]14(63.6)6(33.3)15(65.2)5.0510.098
C3/C4下降[例(%)]a6(33.3)4(30.8)5(23.8)0.5600.795
红细胞沉降率增高[例(%)]a8(47.1)11(68.8)13(68.4)2.1630.354
C反应蛋白＞10 mg/L[例(%)]13(59.1)13(72.2)13(56.5)1.1690.557
肌酶谱增高[例(%)]a11(57.9)10(58.8)7(46.7)0.5850.746
), ArticleFig(id=1211268944141357411, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=EN, label=Tab.2, caption=

Chest CT findings in ILD patients in three groups (n)

, figureFileSmall=null, figureFileBig=null, tableContent=
组别NSIP(n=45)NSIP+OP(n=2)UIP(n=9)OP(n=4)AIP(n=3)
ASS-ILD组(n=22)152410
PM/DM-ILD组(n=18)150111
IPAF组(n=23)150422
), ArticleFig(id=1211268944221049192, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=CN, label=表2, caption=

三组ILD患者胸部CT表现(例)

, figureFileSmall=null, figureFileBig=null, tableContent=
组别NSIP(n=45)NSIP+OP(n=2)UIP(n=9)OP(n=4)AIP(n=3)
ASS-ILD组(n=22)152410
PM/DM-ILD组(n=18)150111
IPAF组(n=23)150422
), ArticleFig(id=1211268944313323885, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=EN, label=Tab.3, caption=

Comparison of myositis autoantibodies in ILD patients of three groups [n(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
抗体类型ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)χ2P
MSA
 抗ARS22(100.0)7(38.9)(1)0(1)(2)–0.000
  Jo-19(40.9)1(5.6)(1)0(1)–0.000
  PL-77(31.8)3(16.7)0(1)–0.007
  PL-123(13.6)2(11.1)0–0.213
  EJ5(22.7)1(5.6)0(1)–0.017
  OJ000––
 非抗ARS6(27.3)9(50.0)11(47.8)2.7520.253
  MDA51(4.5)6(33.3)0(2)–0.001
  SRP04(22.2)1(4.3)–0.031
  Mi-23(13.6)1(5.6)4(17.4)–0.572
  NXP202(11.1)1(4.3)–0.269
  TIF1-γ2(9.1)1(5.6)5(21.7)–0.361
  HMGCR001(4.3)–1.000
  SAE101(5.6)1(4.3)–0.739
MAA16(72.7)12(66.7)15(65.2)0.3220.851
 SSA/Ro15(68.2)11(61.1)10(43.5)2.9640.227
 Ku01(5.6)1(4.3)–0.739
 PM/Scl-1001(4.5)03(13.0)–0.443
 PM/Scl-751(4.5)1(5.6)4(17.4)–0.360
), ArticleFig(id=1211268944418181488, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=CN, label=表3, caption=

三组ILD患者肌炎自身抗体检测结果比较[例(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
抗体类型ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)χ2P
MSA
 抗ARS22(100.0)7(38.9)(1)0(1)(2)–0.000
  Jo-19(40.9)1(5.6)(1)0(1)–0.000
  PL-77(31.8)3(16.7)0(1)–0.007
  PL-123(13.6)2(11.1)0–0.213
  EJ5(22.7)1(5.6)0(1)–0.017
  OJ000––
 非抗ARS6(27.3)9(50.0)11(47.8)2.7520.253
  MDA51(4.5)6(33.3)0(2)–0.001
  SRP04(22.2)1(4.3)–0.031
  Mi-23(13.6)1(5.6)4(17.4)–0.572
  NXP202(11.1)1(4.3)–0.269
  TIF1-γ2(9.1)1(5.6)5(21.7)–0.361
  HMGCR001(4.3)–1.000
  SAE101(5.6)1(4.3)–0.739
MAA16(72.7)12(66.7)15(65.2)0.3220.851
 SSA/Ro15(68.2)11(61.1)10(43.5)2.9640.227
 Ku01(5.6)1(4.3)–0.739
 PM/Scl-1001(4.5)03(13.0)–0.443
 PM/Scl-751(4.5)1(5.6)4(17.4)–0.360
), ArticleFig(id=1211268944531427702, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=EN, label=Tab.4, caption=

The results of antinuclear antibody of ILD patients in three groups (n)

, figureFileSmall=null, figureFileBig=null, tableContent=
抗核抗体结果ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)
阳性(n=44)161117
 高滴度(n=18)3510
 低滴度(n=26)1367
阴性(n=19)676
), ArticleFig(id=1211268944627896701, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=CN, label=表4, caption=

三组ILD患者抗核抗体检测结果(例)

, figureFileSmall=null, figureFileBig=null, tableContent=
抗核抗体结果ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)
阳性(n=44)161117
 高滴度(n=18)3510
 低滴度(n=26)1367
阴性(n=19)676
), ArticleFig(id=1211268944720171393, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=EN, label=Tab.5, caption=

The prognosis of ILD patients in three groups [n(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
转归ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)
好转(n=39)16(72.7)9(50.0)14(60.9)
稳定(n=12)3(13.6)4(22.2)5(21.7)
进展恶化(n=5)2(9.1)1(5.6)2(8.7)
死亡(n=7)1(4.6)4(22.2)2(8.7)
), ArticleFig(id=1211268944820834696, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268934809031655, language=CN, label=表5, caption=

三组ILD患者转归情况[例(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
转归ASS-ILD组(n=22)PM/DM-ILD组(n=18)IPAF组(n=23)
好转(n=39)16(72.7)9(50.0)14(60.9)
稳定(n=12)3(13.6)4(22.2)5(21.7)
进展恶化(n=5)2(9.1)1(5.6)2(8.7)
死亡(n=7)1(4.6)4(22.2)2(8.7)
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63例肌炎自身抗体阳性间质性肺疾病的临床特点分析
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周倩 , 唐霜 , 赵开娅 , 李欣 , 吴彭超 , 唐小葵 *
解放军医学杂志 | 临床研究 2021,46(2): 142-148
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解放军医学杂志 | 临床研究 2021, 46(2): 142-148
63例肌炎自身抗体阳性间质性肺疾病的临床特点分析
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周倩 , 唐霜, 赵开娅, 李欣, 吴彭超, 唐小葵*
作者信息
  • 重庆医科大学附属第一医院呼吸与危重症医学科，重庆 400016

    • 周倩，硕士研究生，主要从事间质性肺疾病的临床研究。E-mail：

通讯作者:

唐小葵，E-mail：
Clinical characteristics analysis of 63 interstitial lung disease patients with positive myositis autoantibodies
Qian Zhou , Shuang Tang, Kai-Ya Zhao, Xin Li, Peng-Chao Wu, Xiao-Kui Tang*
Affiliations
  • Department of Respiratory and Critical Medicine, the First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China
出版时间: 2021-02-28 doi: 10.11855/j.issn.0577-7402.2021.02.06
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摘要
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目的 总结分析肌炎自身抗体阳性间质性肺疾病(ILD)的临床特点，评估肌炎自身抗体检测在ILD临床诊断中的价值。方法 回顾性分析重庆医科大学附属第一医院2018年1月—2019年9月收治的63例结合肌炎自身抗体明确诊断ILD患者的病例资料，分为抗合成酶综合征相关ILD(ASS-ILD)组(n=22)、多发性肌炎/皮肌炎相关ILD(PM/DM-ILD)组(n=18)与自身免疫特征的间质性肺炎(IPAF)组(n=23)，分析对比三组患者的临床特点、治疗及预后情况。结果 ASS-ILD组的雷诺现象、关节症状、技工手、抗ARS抗体阳性率高于IPAF组(P<0.0167)。ASS-ILD组的雷诺现象、咳嗽、呼吸困难、首发表现(呼吸系统症状)、抗ARS抗体阳性率高于PM/DM-ILD组，而发热、肌肉症状、首发表现(皮肤/肌肉症状)阳性率低于PM/DM-ILD组(P<0.0167)。PM/DM-ILD组的肌肉症状、皮肤症状、首发表现(皮肤/肌肉症状)、抗ARS抗体、抗MDA5抗体阳性率高于IPAF组，而首发表现(呼吸系统症状)阳性率低于IPAF组(P<0.0167)。63例患者胸部影像学特征以非特异性间质性肺炎(NSIP)表现为主，肺功能检查结果以弥散障碍及限制性通气障碍为主。87.3%的患者使用激素联合免疫抑制剂治疗。81.0%的患者转归为好转或稳定，19.0%的患者转归为进展恶化或死亡，三组患者转归差异无统计学意义(P>0.05)。结论 ILD患者须重视肌炎自身抗体的筛查，其结果可辅助用于ILD的诊断、分类及预后评估。

肺疾病，间质性  /  抗合成酶综合征  /  多发性肌炎/皮肌炎  /  自身免疫特征的间质性肺炎  /  肌炎自身抗体

Objective To analyze the clinical characteristics of myositis autoantibody detection in the diagnosis of interstitial lung disease (ILD), and explore the diagnosis and treatment of ILD with the help of clinical manifestations combined with antibody results. Methods Sixty-three patients of ILD accurately diagnosed with the help of myositis autoantibody detection were collected retrospectively and divided into three groups: anti-synthetase syndrome associated interstitial lung disease group(ASS-ILD group, n=22), polymyositis/dermatomyositis associated interstitial lung disease group (PM/DM-ILD group, n=18) and interstitial pneumonia with autoimmune features group (IPAF group, n=23). Their clinical characteristics, treatment and prognosis were analyzed and compared. Results The positive rates of Raynaud's phenomenon, joint symptoms, mechanic's hand and anti-ARS antibodies in ASS-ILD group were higher than those in IPAF group (P<0.0167). Compared with PM/DM-ILD group, ASS-ILD group had higher positive rates of Raynaud's phenomenon, cough, dyspnea, respiratory system symptoms as the first manifestation, anti-ARS antibodies but lower positive rates of fever, muscle symptoms, skin/muscle symptoms as the first manifestation (P<0.0167).Compared with IPAF group, PM/DM-ILD group had higher positive rates of muscle symptoms, skin symptoms, skin/muscle symptoms as the first manifestation, anti-ARS antibodies and anti-MDA5 antibody but lower positivity rates of respiratory system symptoms as the first manifestation (P<0.0167). In this study, chest radiological findings of all patients were mainly nonspecific interstitial pneumonia (NSIP). The abnormality in pulmonary functions manifested mainly as diffusion disturbance and restrictive ventilatory dysfunction. 87.3% of all patients were treated with glucocorticoid combined with immunosuppressants. After treatment, 81.0% of these patients showed improvement or stability, while 19.0% showed deterioration or death. The difference in prognosis between the three groups was not statistically significant (P>0.05). Conclusion The detection of myositis autoantibody should be paid more attention in ILD patients. Diagnosis, classification, treatment and prognosis evaluation of ILD could be made with the help of myositis autoantibodies.

lung disease, interstitial  /  antisynthetase syndrome  /  polymyositis/dermatomyositis  /  interstitial pneumonia with autoimmune features  /  myositis autoantibody
周倩, 唐霜, 赵开娅, 李欣, 吴彭超, 唐小葵. 63例肌炎自身抗体阳性间质性肺疾病的临床特点分析. 解放军医学杂志, 2021 , 46 (2) : 142 -148 . DOI: 10.11855/j.issn.0577-7402.2021.02.06
Qian Zhou, Shuang Tang, Kai-Ya Zhao, Xin Li, Peng-Chao Wu, Xiao-Kui Tang. Clinical characteristics analysis of 63 interstitial lung disease patients with positive myositis autoantibodies[J]. Medical Journal of Chinese People’s Liberation Army, 2021 , 46 (2) : 142 -148 . DOI: 10.11855/j.issn.0577-7402.2021.02.06
正文
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间质性肺疾病(interstitial lung disease，ILD)中与结缔组织疾病(connective tissue disease，CTD)相关者占比高达67%，当ILD病因不明时，应积极筛查CTD[1-4]。大多数CTD可根据临床表现结合相应抗体做出诊断，但目前多发性肌炎/皮肌炎(polymyositis/dermatomyositis，PM/DM)多沿用1975年的Bohan/Peter诊断标准[5-6]，主要围绕皮肤和肌肉症状，结合肌电图、肌活检、肌酶谱进行诊断，对无典型皮肤和肌肉表现者或不愿意行肌肉活检者可能造成一定程度的漏诊。近年来，肌炎自身抗体检测越来越受到重视，部分抗体逐渐被纳入PM/DM的诊断标准[7]。肌炎自身抗体分为肌炎特异性自身抗体(myositis specific autoantibody，MSA)和肌炎相关性自身抗体(myositis associated autoantibody，MAA)[4,8-11]。本研究对63例结合肌炎自身抗体结果明确病因诊断的ILD患者的临床特点及疗效进行回顾性分析，以评估肌炎自身抗体检测在ILD诊疗中的意义，探讨如何结合临床表现与抗体检测结果对ILD进行诊断、分类及预后评估。
1 资料与方法
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1.1 研究对象
纳入重庆医科大学附属第一医院2018年1月—2019年9月收治的肌炎自身抗体检测(免疫印迹法)阳性的ILD住院患者63例，均经高分辨率肌炎自身抗体检测(免疫印迹法)结果及临床表现明确诊断。纳入标准：(1)年龄≥18岁；(2)经临床表现、胸部HRCT、肺功能、病理活检确诊ILD；(3)肌炎自身抗体检测结果呈阳性，并结合临床表现和实验室检查可明确ILD病因诊断；(4)病史及临床资料基本完整。排除标准：(1)合并系统性红斑狼疮、类风湿关节炎、系统性硬化、干燥综合征等其他可引起ILD的CTD患者以及合并血管炎的患者；(2)已知病因的ILD患者，如过敏性肺炎、尘肺、毒物、药物、放射性损伤等；(3)合并没有控制的肺部感染；(4)合并恶性肿瘤；(5)合并心肾功能不全；(6)病史及临床资料不全。本研究通过重庆医科大学附属第一医院临床伦理审批及免知情同意申请(2020-294)。
1.2 临床资料
收集患者症状、体征、实验室检查(抗核抗体谱、肌炎自身抗体谱、肌酶谱、红细胞沉降率、C反应蛋白、补体C3、补体C4等)、胸部CT或高分辨率CT、肺功能、治疗后3个月~1年的随访情况。根据诊断标准，将患者分为抗合成酶综合征(anti-synthetase syndrome，ASS)相关ILD(ASS-ILD)组、PM/DM相关ILD(PM/DMILD)组和自身免疫特征的间质性肺炎(interstitial pneumonia with autoimmune features，IPAF)组。根据对CTD-ILD的病情评估，将随访结果中的转归情况分为好转、稳定、进展恶化、死亡四类。
1.3 诊断标准
ASS的诊断标准：2011年Solomon等提出两项主要标准：(1)不明原因的ILD；(2)符合Bohan/Peter诊断标准的PM或DM。三项次要标准：(1)关节炎；(2)雷诺现象；(3)技工手。若存在抗ARS抗体，并满足“两项主要标准”或“一项主要+两项次要标准”，即可诊断ASS[12-13]。PM/DM依据1975年的Bohan/Peter标准进行诊断[5-6]。IPAF依据2015年ATS/ERS官方共识[14]进行诊断。在遵循以上诊断标准的基础上，我院包括呼吸内科、风湿免疫科、影像科在内的CTD-ILD多学科会诊团队对ASS-ILD、PM/DM-ILD、IPAF进行了分类诊断。根据患者的生活质量、肺功能、影像学表现、治疗可逆性、纤维化进展风险、CTD活动度等对CTD-ILD进行了病情评估[2]。CTD-ILD胸部影像学分类参考2013年ATS/ERS提出的特发性间质性肺炎影像学分类标准[2,15]，并分别由我院多学科会诊团队中有CTD-ILD胸部影像学诊断经验的呼吸科、放射科医师独立阅片，意见不统一时经协商决定。
1.4 统计学处理
采用SPSS 22.0软件进行统计分析。计数资料以例(%)表示，组间比较采用χ2检验或Fisher确切概率法。计量资料若符合正态分布，则以$\bar{x}±s$表示，若不符合，则以M(Q1，Q3)表示。计量资料若同时满足正态性和方差齐性，多组间比较采用单因素方差分析，否则采用秩和检验。多组间两两比较采用Bonferroni法调整检验水准。P<0.0167为差异有统计学意义。
2 结果
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2.1 一般情况
共纳入63例结合肌炎自身抗体明确诊断的ILD患者，分为ASS-ILD组(34.9%，22/63)、PM/DM-ILD组(28.6%，18/63)与IPAF组(36.5%，23/63)。
2.2 临床特征
2.2.1 症状与体征
首发表现、发热、皮肤症状、肌肉症状、关节症状、雷诺现象、技工手、咳嗽、呼吸困难等的阳性率在三组间差异有统计学意义(P<0.05)。进一步两两比较显示，ASS-ILD组雷诺现象、关节症状、技工手的阳性率高于IPAF组，雷诺现象、咳嗽、呼吸困难的阳性率高于PM/DMILD组，但发热、肌肉症状的阳性率低于PM/DMILD组；PM/DM-ILD组的肌肉症状、皮肤症状阳性率高于IPAF组(P<0.05)。ASS-ILD组和IPAF组主要以呼吸系统症状为首发表现，而PM/DM-ILD组主要以皮肤/肌肉症状为首发表现(表1)。
2.2.2 肺功能特征
肺功能检查结果显示，在63例患者中，弥散障碍以及限制性通气功能障碍为主要异常情况。
2.2.3 胸部CT特征
63例患者中，45例(71.4%)表现为非特异性间质性肺炎(non-specific interstitial pneumonia，NSIP)，2例(3.2%)表现为NSIP合并机化性肺炎(organizing pneumonia，OP)，9例(14.3%)表现为普通型间质性肺炎(usual interstitial pneumonia，UIP)，4例(6.3%)表现为OP，3例(4.8%)表现为急性间质性肺炎(acute interstitial pneumonia，AIP)(表2，图1)。
2.3 抗体情况
2.3.1 肌炎自身抗体
ASS-ILD组抗ARS抗体阳性率为100%，以抗Jo-1、抗PL-7抗体多见，非抗ARS抗体阳性率为27.3%，MAA阳性率为72.7%。PM/DM-ILD组抗ARS抗体阳性率为38.9%，以抗PL-7、抗PL-12抗体多见，非抗ARS抗体阳性率为50.0%，以抗MDA5、抗SRP抗体多见，MAA阳性率为66.7%。IPAF组中抗ARS抗体均为阴性，非抗ARS抗体阳性率为47.8%，以抗Mi-2、抗TIF1-γ抗体多见，MAA阳性率为65.2%。各组MAA均以抗SSA/Ro抗体多见。抗ARS抗体阳性率在三组间差异有统计学意义(P<0.05)，非抗ARS抗体及MAA阳性率在三组间差异无统计学意义(P>0.05，表3)。
2.3.2 抗核抗体
63例患者均完成抗核抗体谱检测，抗核抗体结果显示，19例(30.2%)为阴性，44例(69.8%)为阳性。抗核抗体高滴度仅有18例(40.9%)，其余26例(59.1%)呈低滴度。抗核抗体滴度在各组间的差异无统计学意义(H=2.167，P=0.338，表4)。
2.4 治疗与随访
55例(87.3%)患者使用激素联合环磷酰胺、硫酸羟氯喹、环孢素、甲氨蝶呤、雷公藤片、硫唑嘌呤、吗替麦考酚酯等免疫抑制剂治疗，其中13例(23.6%)同时联合丙种球蛋白治疗。63例患者中，4例在院死亡，59例治疗后出院并随访。其中3例患者于随访过程中死亡。三组患者转归差异无统计学意义(H=2.537，P=0.281)。总体上，治疗后81.0%的患者转归为好转或稳定(表5，图2)，19.0%的患者转归为进展恶化或死亡。
3 讨论
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本研究发现，不同类型CTD-ILD主要的累及脏器及临床特征有所不同，其中ASS-ILD以呼吸系统症状、关节症状、雷诺现象、技工手为主要特征，PM/DM-ILD以皮肤、肌肉受累较重，IPAF以呼吸系统症状为主。
本研究肌炎自身抗体检测结果显示，22例ASSILD患者的抗ARS抗体均为阳性，除无抗OJ抗体阳性患者外，抗Jo-1、抗PL-7、抗PL-12、抗EJ抗体均有一定的阳性比例，其中Jo-1抗体阳性率为40.9%。既往研究报道，ASS-ILD中抗Jo-1抗体阳性率为35%~44%，虽然抗Jo-1抗体是ASS-ILD最常见的抗ARS抗体，但非抗Jo-1的ARS抗体也占有较高比例[12,17]。18例PM/DM-ILD患者肌炎自身抗体检测结果显示，抗ARS抗体阳性率为38.9%，非抗ARS抗体阳性率为50%，其中以抗MDA5、抗SRP抗体多见，6例抗MDA5抗体阳性患者中死亡3例(50.0%)，考虑与抗MDA5抗体阳性者通常对激素治疗反应差且常出现快速进展的间质性肺病有关[11,18-20]。在23例IPAF患者中，非抗ARS抗体阳性率为47.8%，其中以抗Mi-2、抗TIF1-γ抗体较多见，MAA阳性率为65.2%，以抗SSA/Ro抗体较多见。因此，笔者认为，肌炎自身抗体特别是MSA是疾病诊断、分类及预后评估的重要工具[4,21-22]。
另外，63例患者中，仅有18例ANA呈高滴度阳性，且结合ANA谱无法明确诊断。考虑到常规自身抗体检测无法完全明确ILD的诊断[1]，因此无论ANA结果如何，均有必要行肌炎自身抗体检测。
本研究患者的胸部影像学特征以NSIP表现为主，符合CTD-ILD特点[1,14,23]，少部分患者表现为NSIP+OP混合型。有3例表现为AIP，其中1例IPAF患者经治疗好转，1例IPAF患者死于重症感染，1例PM/DM-ILD患者死于呼吸衰竭。
本研究中，大部分患者采用激素联合免疫抑制剂治疗，经3个月至1年的随访，81.0%的患者转归为好转或稳定，19.0%的患者转归为进展恶化或死亡。63例中共死亡7例(11.1%)，分别为1例ASSILD，4例PM/DM-ILD，2例IPAF，死亡原因主要为呼吸衰竭、合并重症感染、不配合或放弃治疗等。整体而言大部分患者经过治疗病情在短期内好转或稳定，虽然PM/DM-ILD及IPAF组好转或稳定的比例稍低于ASS-ILD组，但三组间转归差异无统计学意义(P>0.05)，这可能与本研究样本量不大有一定关系。在今后的研究中需扩大样本量、延长随访时间，以进一步观察各种类型的转归情况。
综上所述，肌炎自身抗体检测对ILD的病因诊断、分类、预后评估具有重要意义，对病因不明的ILD患者，无论是否存在皮肤肌肉的表现均应进行肌炎自身抗体筛查，从而做到早诊断、早评估、早治疗，让患者尽早获益。
基金
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  • 重庆市科卫联合医学科研项目(2020MSXM033)
文献
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2021年第46卷第2期
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doi: 10.11855/j.issn.0577-7402.2021.02.06
  • 接收时间：2020-03-21
  • 首发时间：2025-12-26
  • 出版时间：2021-02-28
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  • 收稿日期：2020-03-21
  • 修回日期：2020-07-27
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Project of Chongqing Science and Health Joint Medical Research(2020MSXM033)
重庆市科卫联合医学科研项目(2020MSXM033)
作者信息
    重庆医科大学附属第一医院呼吸与危重症医学科，重庆 400016

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