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Article(id=1211268823173427366, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1211268819788632695, articleNumber=null, orderNo=null, doi=10.11855/j.issn.0577-7402.2021.01.07, pmid=null, cstr=null, oa=null, hot=null, price=null, onlineType=0, articleFormat=0, articleType=null, articleTypeStr=research-article, receivedDate=1591113600000, receivedDateStr=2020-06-03, revisedDate=1606838400000, revisedDateStr=2020-12-02, acceptedDate=null, acceptedDateStr=null, onlineDate=1766718591731, onlineDateStr=2025-12-26, pubDate=1611763200000, pubDateStr=2021-01-28, doiRegisterDate=null, doiRegisterDateStr=null, onlineIssueDate=1766718591731, onlineIssueDateStr=2025-12-26, onlineJustAcceptDate=null, onlineJustAcceptDateStr=null, onlineFirstDate=null, onlineFirstDateStr=null, sourceXml=null, magXml=null, createTime=1766718591731, creator=13701087609, updateTime=1766718591731, updator=13701087609, issue=Issue{id=1211268819788632695, tenantId=1146029695717560320, journalId=1189873630562394117, year='2021', volume='46', issue='1', pageStart='1', pageEnd='100', issueExtLink='null', onlineDate='null', pubDate='null', beforeIssueId=null, nextIssueId=null, price=null, status=1, issueComplete=1, articleOrder=1, issueType=-1, specialIssue=0, createTime=1766718590924, creator=13701087609, updateTime=1766718828068, updator=13701087609, preIssue=null, nextIssue=null, ext={EN=IssueExt(id=1211269814484594852, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1211268819788632695, language=EN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=), CN=IssueExt(id=1211269814484594853, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1211268819788632695, language=CN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=)}, issueFiles=null}, startPage=36, endPage=41, ext={EN=ArticleExt(id=1211268823508971691, articleId=1211268823173427366, tenantId=1146029695717560320, journalId=1189873630562394117, language=EN, title=Clinical characteristics analysis of primary biliary cholangitis with features of autoimmune hepatitis, 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 178 primary biliary cholangitis (PBC) patients with features of autoimmune hepatitis (AIH). Methods Four hundred and sixty-one PBC patients diagnosed in the Department of Gastroenterology, Xijing Hospital Affiliated to Air Force Medical University from December 2008 to December 2018 were included in this study. According to the diagnostic criteria of PBC-AIH, AIH-PBC and PBC, they were divided into PBC-AIH OS group(n=50), AIH-PBC group (n=178) and pure PBC group (n=233). Comparison of general clinical symptoms of three groups (jaundice, fatigue, pruritus, etc.); biochemical and immunological indices [alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), gamma glutamyltranspeptidase (GGT), immunoglobulin G/M (IgG, IgM), total/direct bilirubin (TBIL, DBIL), albumin/globulin (ALB, GLB)]; autoantibodies [antinuclear antibody (ANA), antimitochondrial antibody (AMA), anti-smooth muscle antibody (ASMA)]; stage and grade of the inflammation and fibrosis of the liver, noninvasive fibrosis score of fibrosis index based on the four factors (FIB-4) and aspartate aminotransferase/platelet ratio index (APRI); 5-year cumulative incidence of adverse events. Results The incidence of jaundice and inappetence in AIH-PBC group and PBC group was significantly lower than that in PBC-AIH OS group (P<0.05). The differences of ALT, AST, TBIL, IgG, IgM and GLB between the groups were statistically significant (P<0.05), and AIH-PBC group and PBC-AIH OS group were mostly higher than pure PBC group. Autoantibody detection showed that the positivity rates of SSA and Ro-52 antibodies in AIH-PBC group were significantly higher than those in pure PBC group, while the positivity rates of ASMA, soluble liver antigen/liver pancreatic antigen antibody (SLA/LP) and SSB were significantly lower than those in PBC-AIH OS group (P<0.05). The IgG and IgM of AIH-PBC group were significantly higher than those of pure PBC group (P<0.05). The liver histopathological stage, FIB-4 and APRI indexes of AIH-PBC group and PBC-AIH OS group were significantly higher than those of pure PBC group (P<0.05). The 5-year cumulative adverse event incidence of PCB-AIH OS group was significantly higher than that of AIH-PBC group, and that of AIH-PBC group was significantly higher than that of pure PBC group (P<0.05). Conclusions The clinical manifestations of AIH-PBC patients are significantly different from those of PBCAIH OS and pure PBC patients, and their long-term prognosis is worse than that of pure PBC patients.

, correspAuthors=Ying Han, authorNote=null, correspAuthorsNote=
*E-mail:
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目的 分析伴自身免疫性肝炎(AIH)特征的原发性胆汁性胆管炎(PBC)患者的临床特征。方法 纳入2008年12月—2018年12月空军军医大学附属西京医院消化内科确诊的PBC患者461例，根据PBC-AIH重叠综合征(PBC-AIH OS)、AIH-PBC及PBC的诊断标准分为PBC-AIH OS组(n=50)、AIH-PBC组(n=178)与单纯PBC组(n=233)，分析各组一般临床症状(黄疸、乏力、瘙痒等)、生化免疫指标[谷丙转氨酶(ALT)、谷草转氨酶(AST)、碱性磷酸酶(ALP)、γ-谷氨酰转肽酶(GGT)、免疫球蛋白G/M(IgG、IgM)、总/直接胆红素(TBIL、DBIL)、白/球蛋白(ALB、GLB)]、自身抗体[抗核抗体(ANA)、抗线粒体抗体(AMA)、抗平滑肌抗体(ASMA)]、肝脏炎症纤维化分级分期、基于4因子的纤维化指数(FIB-4)和天门冬氨酸氨基转移酶/血小板比值指数(APRI)，以及5年不良事件累积发生率等。结果 AIH-PBC组及单纯PBC组黄疸、纳差发生率均明显低于PBC-AIH OS组(P<0.05)。各组ALT、AST、TBIL、IgG、IgM、GLB水平差异有统计学意义(P<0.05)，多以AIH-PBC组及PBC-AIH OS组较高。自身抗体检测结果显示，AIH-PBC组SSA、Ro-52抗体检出率明显高于单纯PBC组，ASMA、可溶性肝抗原/肝胰抗原抗体(SLA/LP)、SSB检出率明显低于PBC-AIH OS组(P<0.05)。AIH-PBC组IgG、IgM水平高于单纯PBC组(P<0.05)。AIH-PBC组和PBC-AIH OS组肝组织病理分级分期及FIB-4、APRI指数明显高于单纯PBC组(P<0.05)。5年累积不良事件发生率PBC-AIH OS组明显高于AIH-PBC组，AIH-PBC组明显高于单纯PBC组(P<0.05)。结论 AIH-PBC患者在临床表现上与PBC-AIH OS及单纯PBC患者有较大差异，长期预后较单纯PBC患者差。

, correspAuthors=韩英, authorNote=null, correspAuthorsNote=
韩英，E-mail：
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杨宁，硕士研究生，主要从事自身免疫性肝病临床诊疗方面的研究。E-mail：

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杨宁，硕士研究生，主要从事自身免疫性肝病临床诊疗方面的研究。E-mail：

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Rheum Dis Clin North Am, 2018, 44(1): 65-87., articleTitle=Rheumatic manifestations in autoimmune liver disease, refAbstract=null)], funds=[Fund(id=1211268829724930508, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, awardId=81770569, language=EN, fundingSource=National Natrual Science Foundation of China(81770569), fundOrder=null, country=null), Fund(id=1211268829817205202, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, awardId=81770569, language=CN, fundingSource=国家自然科学基金(81770569), fundOrder=null, country=null)], companyList=[AuthorCompany(id=1211268824712736982, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, xref=null, ext=[AuthorCompanyExt(id=1211268824721125591, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, companyId=1211268824712736982, language=EN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=Department of Gastroenterology, Xijing Hospital Affiliated to Air Force Medical University, Xi'an 710032, China), AuthorCompanyExt(id=1211268824729514200, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, companyId=1211268824712736982, language=CN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=空军军医大学附属西京医院消化内科，西安 710032)])], figs=[ArticleFig(id=1211268828584079759, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=EN, label=Fig.1, caption=APRI, FIB-4 indexes and pathological fibrosis grade and inflammatory stage of liver tissue, figureFileSmall=W9INdE9ek6kECuEqCPmVZQ==, figureFileBig=vfYANxEcH321SkJXAJCkiw==, tableContent=null), ArticleFig(id=1211268828730880404, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=CN, label=图1, caption=各组原发性胆汁性胆管炎APRI、FIB-4指数及肝组织病理纤维化分级和炎症分期

与PBC-AIH OS组比较，(1)P<0.05；与单纯PBC组比较，(2)P<0.05。

, figureFileSmall=W9INdE9ek6kECuEqCPmVZQ==, figureFileBig=vfYANxEcH321SkJXAJCkiw==, tableContent=null), ArticleFig(id=1211268828936401316, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=EN, label=Fig.2, caption=5-year cumulative incidence of adverse events in each group, figureFileSmall=9bSxStsdpc4U/gsIymWeUA==, figureFileBig=QqAScswbDM/21qkNGkS+Dg==, tableContent=null), ArticleFig(id=1211268829020287399, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=CN, label=图2, caption=各组原发性胆汁性胆管炎患者5年累积不良事件发生率, figureFileSmall=9bSxStsdpc4U/gsIymWeUA==, figureFileBig=QqAScswbDM/21qkNGkS+Dg==, tableContent=null), ArticleFig(id=1211268829112562095, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=EN, label=Tab.1, caption=

Comparison of the incidence of clinical symptoms in each group [n(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
症状PBC-AIH OS组(n=50)AIH-PBC组(n=178)单纯PBC组(n=233)χ2P
乏力30(60.0)98(55.1)113(48.5)3.0810.214
瘙痒13(26.0)54(30.3)54(23.2)2.6830.263
口干眼干26(52.0)79(44.4)87(37.3)4.5370.104
关节疼痛3(6.0)16(9.0)13(5.6)1.8240.432
黄疸14(28.0)22(12.4)(1)25(10.7)(1)10.8970.004
腹胀19(38.0)62(34.8)62(26.6)4.4740.107
纳差21(42.0)43(24.2)(1)54(23.2)(1)7.9890.019
), ArticleFig(id=1211268829225808307, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=CN, label=表1, caption=

各组原发性胆汁性胆管炎临床症状发生率比较[例(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
症状PBC-AIH OS组(n=50)AIH-PBC组(n=178)单纯PBC组(n=233)χ2P
乏力30(60.0)98(55.1)113(48.5)3.0810.214
瘙痒13(26.0)54(30.3)54(23.2)2.6830.263
口干眼干26(52.0)79(44.4)87(37.3)4.5370.104
关节疼痛3(6.0)16(9.0)13(5.6)1.8240.432
黄疸14(28.0)22(12.4)(1)25(10.7)(1)10.8970.004
腹胀19(38.0)62(34.8)62(26.6)4.4740.107
纳差21(42.0)43(24.2)(1)54(23.2)(1)7.9890.019
), ArticleFig(id=1211268829330665911, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=EN, label=Tab.2, caption=

Comparison of the baseline data of laboratory indicators in each groups [M(P25, P75)]

, figureFileSmall=null, figureFileBig=null, tableContent=
指标PBC-AIH OS组(n=50)AIH-PBC组(n=178)单纯PBC组(n=233)ZP
ALT(U/L)112.0(62.8，220.5)57.0(38.8，93.8)(1)57.0(34.0，96.0)31.865<0.001
AST(U/L)137.5(87.0，251.0)64.0(46.8，100.3)(1)56.0(38.0，86.0)60.623<0.001
TBIL(mmol/L)26.6(15.4，52.2)20.6(12.6，36.9)(2)16.0(11.5，25.9)18.558<0.001
DBIL(mmol/L)13.6(7.2，40.9)9.3(5.0，23.8)(1)(2)6.0(4.2，10.5)36.129<0.001
ALP(U/L)273.5(153.8，457.5)304.0(178.3，490.5)255.0(152.0，439.0)3.3100.191
GGT(U/L)309.0(118.3，466.0)274.5(133.8，540.5)285.5(117.6，480.3)0.3900.823
IgG(g/L)28.4(16.1，34.4)17.7(15.3，20.2)(1)(2)13.4(11.3，14.8)125.809<0.001
IgM(g/L)4.3(2.4，5.9)3.6(2.3，5.4)(2)3.0(1.8，4.5)12.4820.002
ALB(g/L)36.2(31.6，38.6)38.7(35.5，41.6)(1)(2)39.9(37.3，42.3)34.757<0.001
GLB(g/L)44.9(32.7，51.1)34.2(30.4，39.1)(1)(2)29.3(26.7，31.7)108.522<0.001
), ArticleFig(id=1211268829439717822, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=CN, label=表2, caption=

各组原发性胆汁性胆管炎生化免疫指标水平比较[M(P25, P75)]

, figureFileSmall=null, figureFileBig=null, tableContent=
指标PBC-AIH OS组(n=50)AIH-PBC组(n=178)单纯PBC组(n=233)ZP
ALT(U/L)112.0(62.8，220.5)57.0(38.8，93.8)(1)57.0(34.0，96.0)31.865<0.001
AST(U/L)137.5(87.0，251.0)64.0(46.8，100.3)(1)56.0(38.0，86.0)60.623<0.001
TBIL(mmol/L)26.6(15.4，52.2)20.6(12.6，36.9)(2)16.0(11.5，25.9)18.558<0.001
DBIL(mmol/L)13.6(7.2，40.9)9.3(5.0，23.8)(1)(2)6.0(4.2，10.5)36.129<0.001
ALP(U/L)273.5(153.8，457.5)304.0(178.3，490.5)255.0(152.0，439.0)3.3100.191
GGT(U/L)309.0(118.3，466.0)274.5(133.8，540.5)285.5(117.6，480.3)0.3900.823
IgG(g/L)28.4(16.1，34.4)17.7(15.3，20.2)(1)(2)13.4(11.3，14.8)125.809<0.001
IgM(g/L)4.3(2.4，5.9)3.6(2.3，5.4)(2)3.0(1.8，4.5)12.4820.002
ALB(g/L)36.2(31.6，38.6)38.7(35.5，41.6)(1)(2)39.9(37.3，42.3)34.757<0.001
GLB(g/L)44.9(32.7，51.1)34.2(30.4，39.1)(1)(2)29.3(26.7，31.7)108.522<0.001
), ArticleFig(id=1211268829531992515, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=EN, label=Tab.3, caption=

Comparison of antibody positive rate in each group [n(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
指标PBC-AIH OS组(n=50)AIH-PBC组(n=178)单纯PBC组(n=233)χ2P
SSA10(20.0)30(16.9)(2)20(8.6)8.511*0.014
SSB7(14.0)7(3.9)(1)6(2.6)13.062*0.001
ANA48(96.0)169(94.9)211(90.6)3.763*0.152
AMA/M241(82.0)164(92.1)(1)217(93.1)6.725*0.035
ASMA10(20.0)2(1.1)(1)8(3.4)22.912*<0.001
Sp1005(10.0)17(9.6)25(10.7)0.169*0.925
gp21021(42.0)66(37.1)75(32.2)2.225*0.330
LC-11(2.0)2(1.1)04.051Ñ0.081
Ro-5221(42.0)88(49.4)(2)67(28.8)18.640*<0.001
SLA/LP4(8.0)1(0.6)(1)1(0.4)11.044Ñ0.002
), ArticleFig(id=1211268829620072904, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1211268823173427366, language=CN, label=表3, caption=

各组原发性胆汁性胆管炎抗体阳性率比较[例(%)]

, figureFileSmall=null, figureFileBig=null, tableContent=
指标PBC-AIH OS组(n=50)AIH-PBC组(n=178)单纯PBC组(n=233)χ2P
SSA10(20.0)30(16.9)(2)20(8.6)8.511*0.014
SSB7(14.0)7(3.9)(1)6(2.6)13.062*0.001
ANA48(96.0)169(94.9)211(90.6)3.763*0.152
AMA/M241(82.0)164(92.1)(1)217(93.1)6.725*0.035
ASMA10(20.0)2(1.1)(1)8(3.4)22.912*<0.001
Sp1005(10.0)17(9.6)25(10.7)0.169*0.925
gp21021(42.0)66(37.1)75(32.2)2.225*0.330
LC-11(2.0)2(1.1)04.051Ñ0.081
Ro-5221(42.0)88(49.4)(2)67(28.8)18.640*<0.001
SLA/LP4(8.0)1(0.6)(1)1(0.4)11.044Ñ0.002
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伴自身免疫性肝炎特征的原发性胆汁性胆管炎的临床特征
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杨宁 , 孙可帅 , 田思远 , 刘雁声 , 贾桂 , 韩英 *
解放军医学杂志 | 临床研究 2021,46(1): 36-41
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解放军医学杂志 | 临床研究 2021, 46(1): 36-41
伴自身免疫性肝炎特征的原发性胆汁性胆管炎的临床特征
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杨宁 , 孙可帅, 田思远, 刘雁声, 贾桂, 韩英*
作者信息
  • 空军军医大学附属西京医院消化内科，西安 710032

    • 杨宁，硕士研究生，主要从事自身免疫性肝病临床诊疗方面的研究。E-mail：

通讯作者:

韩英，E-mail：
Clinical characteristics analysis of primary biliary cholangitis with features of autoimmune hepatitis
Ning Yang , Ke-Shuai Sun, Si-Yuan Tian, Yan-Sheng Liu, Gui Jia, Ying Han*
Affiliations
  • Department of Gastroenterology, Xijing Hospital Affiliated to Air Force Medical University, Xi'an 710032, China
出版时间: 2021-01-28 doi: 10.11855/j.issn.0577-7402.2021.01.07
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摘要
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目的 分析伴自身免疫性肝炎(AIH)特征的原发性胆汁性胆管炎(PBC)患者的临床特征。方法 纳入2008年12月—2018年12月空军军医大学附属西京医院消化内科确诊的PBC患者461例，根据PBC-AIH重叠综合征(PBC-AIH OS)、AIH-PBC及PBC的诊断标准分为PBC-AIH OS组(n=50)、AIH-PBC组(n=178)与单纯PBC组(n=233)，分析各组一般临床症状(黄疸、乏力、瘙痒等)、生化免疫指标[谷丙转氨酶(ALT)、谷草转氨酶(AST)、碱性磷酸酶(ALP)、γ-谷氨酰转肽酶(GGT)、免疫球蛋白G/M(IgG、IgM)、总/直接胆红素(TBIL、DBIL)、白/球蛋白(ALB、GLB)]、自身抗体[抗核抗体(ANA)、抗线粒体抗体(AMA)、抗平滑肌抗体(ASMA)]、肝脏炎症纤维化分级分期、基于4因子的纤维化指数(FIB-4)和天门冬氨酸氨基转移酶/血小板比值指数(APRI)，以及5年不良事件累积发生率等。结果 AIH-PBC组及单纯PBC组黄疸、纳差发生率均明显低于PBC-AIH OS组(P<0.05)。各组ALT、AST、TBIL、IgG、IgM、GLB水平差异有统计学意义(P<0.05)，多以AIH-PBC组及PBC-AIH OS组较高。自身抗体检测结果显示，AIH-PBC组SSA、Ro-52抗体检出率明显高于单纯PBC组，ASMA、可溶性肝抗原/肝胰抗原抗体(SLA/LP)、SSB检出率明显低于PBC-AIH OS组(P<0.05)。AIH-PBC组IgG、IgM水平高于单纯PBC组(P<0.05)。AIH-PBC组和PBC-AIH OS组肝组织病理分级分期及FIB-4、APRI指数明显高于单纯PBC组(P<0.05)。5年累积不良事件发生率PBC-AIH OS组明显高于AIH-PBC组，AIH-PBC组明显高于单纯PBC组(P<0.05)。结论 AIH-PBC患者在临床表现上与PBC-AIH OS及单纯PBC患者有较大差异，长期预后较单纯PBC患者差。

原发性胆汁性胆管炎  /  肝炎，自身免疫性  /  临床特征

Objective To analyze the clinical characteristics of 178 primary biliary cholangitis (PBC) patients with features of autoimmune hepatitis (AIH). Methods Four hundred and sixty-one PBC patients diagnosed in the Department of Gastroenterology, Xijing Hospital Affiliated to Air Force Medical University from December 2008 to December 2018 were included in this study. According to the diagnostic criteria of PBC-AIH, AIH-PBC and PBC, they were divided into PBC-AIH OS group(n=50), AIH-PBC group (n=178) and pure PBC group (n=233). Comparison of general clinical symptoms of three groups (jaundice, fatigue, pruritus, etc.); biochemical and immunological indices [alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), gamma glutamyltranspeptidase (GGT), immunoglobulin G/M (IgG, IgM), total/direct bilirubin (TBIL, DBIL), albumin/globulin (ALB, GLB)]; autoantibodies [antinuclear antibody (ANA), antimitochondrial antibody (AMA), anti-smooth muscle antibody (ASMA)]; stage and grade of the inflammation and fibrosis of the liver, noninvasive fibrosis score of fibrosis index based on the four factors (FIB-4) and aspartate aminotransferase/platelet ratio index (APRI); 5-year cumulative incidence of adverse events. Results The incidence of jaundice and inappetence in AIH-PBC group and PBC group was significantly lower than that in PBC-AIH OS group (P<0.05). The differences of ALT, AST, TBIL, IgG, IgM and GLB between the groups were statistically significant (P<0.05), and AIH-PBC group and PBC-AIH OS group were mostly higher than pure PBC group. Autoantibody detection showed that the positivity rates of SSA and Ro-52 antibodies in AIH-PBC group were significantly higher than those in pure PBC group, while the positivity rates of ASMA, soluble liver antigen/liver pancreatic antigen antibody (SLA/LP) and SSB were significantly lower than those in PBC-AIH OS group (P<0.05). The IgG and IgM of AIH-PBC group were significantly higher than those of pure PBC group (P<0.05). The liver histopathological stage, FIB-4 and APRI indexes of AIH-PBC group and PBC-AIH OS group were significantly higher than those of pure PBC group (P<0.05). The 5-year cumulative adverse event incidence of PCB-AIH OS group was significantly higher than that of AIH-PBC group, and that of AIH-PBC group was significantly higher than that of pure PBC group (P<0.05). Conclusions The clinical manifestations of AIH-PBC patients are significantly different from those of PBCAIH OS and pure PBC patients, and their long-term prognosis is worse than that of pure PBC patients.

primary biliary cholangitis  /  hepatitis, autoimmune  /  clinical characteristics
杨宁, 孙可帅, 田思远, 刘雁声, 贾桂, 韩英. 伴自身免疫性肝炎特征的原发性胆汁性胆管炎的临床特征. 解放军医学杂志, 2021 , 46 (1) : 36 -41 . DOI: 10.11855/j.issn.0577-7402.2021.01.07
Ning Yang, Ke-Shuai Sun, Si-Yuan Tian, Yan-Sheng Liu, Gui Jia, Ying Han. Clinical characteristics analysis of primary biliary cholangitis with features of autoimmune hepatitis[J]. Medical Journal of Chinese People’s Liberation Army, 2021 , 46 (1) : 36 -41 . DOI: 10.11855/j.issn.0577-7402.2021.01.07
正文
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原发性胆汁性胆管炎(primary biliary cholangitis，PBC)和自身免疫性肝炎(autoimmune hepatitis，AIH)均为自身免疫介导的肝胆炎症损伤性疾病，两者在临床表现、生物化学、血清学、病理学等方面各有特点，治疗和预后亦不同[1]。部分患者可同时具有这两种疾病的特征，称为PBC-AIH重叠综合征(PBC-AIH OS)。相比单纯PBC或AIH患者，PBCAIH OS患者疾病进展较快，发生肝硬化不良事件的风险较高[2-4]。采用熊去氧胆酸(ursodeoxycholic acid，UDCA)联合糖皮质激素治疗可使PBC-AIH OS患者生化应答率明显升高，并可延缓甚至逆转其组织学进展[5]。巴黎标准[6]作为一种较为有效的诊断方法，目前已被欧洲肝脏研究学会(EASL)及美国肝病研究协会(AASLD)采纳作为诊断PBC-AIH OS的推荐方法[7-8]。然而有研究发现，在中国人群中使用巴黎标准诊断PBC-AIH OS虽特异度较高，但敏感度较低[9]。临床上部分PBC患者会伴随AIH特征，如转氨酶、IgG升高，组织学表现为界面性肝炎，但依据巴黎标准无法诊断为PBC-AIH OS。这类患者被称为伴AIH特征的PBC(AIH-PBC)，其临床表现及长期预后是否与PBC-AIH OS及单纯PBC患者有所差异鲜有报道。本研究旨在分析AIH-PBC患者的临床表现及长期预后。
1 资料与方法
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1.1 研究对象及分组
纳入2008年12月—2018年12月于空军军医大学附属西京医院消化内科确诊的461例PBC患者，根据相应诊断标准分为PBCAIH OS组(n=50)、AIH-PBC组(n=178)与单纯PBC组(n=233)。
1.2 诊断标准
PBC及PBC-AIH OS的诊断参照2017年EASL的《原发性胆汁性胆管炎诊疗指南》[7]，其中PBC-AIH OS的诊断需同时符合以下各自诊断指标中的2项或3项。(1)PBC诊断指标：①ALP≥2倍正常值上限(ULN)或GGT≥5×ULN；②抗线粒体抗体(AMA)/AMA-M2阳性；③组织学可见特征性胆管损害。(2)AIH诊断指标：①ALT≥5×ULN；②IgG≥2×ULN或抗平滑肌抗体(ASMA)阳性；③组织学表现为中重度界面性肝炎(此项必须满足)。AIH-PBC的界定使用国际自免肝小组(IAIHG)简化积分系统[10]。简化积分<6分为单纯PBC；简化积分≥6分为AIH-PBC。肝硬化不良事件是指随访过程中出现的腹水、上消化道出血、肝性脑病、肝肾综合征、自发性腹膜炎、肝癌、肝移植、死亡等事件。所有病例基线资料完整，并排除病毒性肝炎、酒精性肝病、遗传代谢性肝病、药物性肝损害、肝移植术后等。
1.3 观察指标
对比分析各组患者的一般临床症状(黄疸、乏力、瘙痒等)、生化免疫指标[谷丙转氨酶(ALT)、谷草转氨酶(AST)、碱性磷酸酶(ALP)、γ-谷氨酰转肽酶(GGT)、免疫球蛋白G/M(IgG、IgM)、总/直接胆红素(TBIL、DBIL)、白/球蛋白(ALB、GLB)]、自身抗体[抗核抗体(ANA)、抗线粒体抗体(AMA)、抗平滑肌抗体(ASMA)、可溶性肝抗原/肝胰抗原抗体(SLA/LP)、SSA抗体、SSB抗体、Ro-52抗体、Sp100抗体、gp210抗体、抗肝细胞胞质/型抗体(LC-1)等]、肝组织病理纤维化分级和炎症分期、基于4因子的纤维化指数(FIB-4)和天门冬氨酸氨基转移酶/血小板比值指数(APRI)[11-12]，以及5年不良事件累积发生率等。组织学采用慢性肝炎分级分期标准[13]，炎症程度分为0~4级，纤维化程度分为0~4期。
1.4 统计学处理
采用SPSS 23.0软件进行统计分析。所得数据进行正态性检验，其中正态分布的计量资料以$\bar{x}±s$表示，组间比较采用方差分析，进一步两两比较采用Dunnett-t检验；偏态分布的计量资料以M(P25, P75)表示，组间比较采用非参数秩和检验，进一步两两比较联合Bonferroni法校正P值。计数资料以率(%)表示，组间比较采用χ2检验或Fisher精确检验。P<0.05为差异有统计学意义。
2 结果
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2.1 一般资料及症状
AIH-PBC组男24例，女154例，年龄(51.1±9.7)岁；PBC-AIH OS组男4例，女46例，年龄(51.5±9.0)岁；单纯PBC组男35例，女198例，年龄(50.8±9.3)岁。各组患者性别、年龄差异无统计学意义(P>0.05)。PBC-AIH OS组黄疸、纳差发生率为28.0%、42.0%，高于AIH-PBC组(12.4%、24.2%)及单纯PBC组(10.7%、23.2%)，差异有统计学意义(P<0.05)；各组乏力、瘙痒、口干眼干、关节疼痛、腹胀等症状发生率差异无统计学意义(P>0.05，表1)。
2.2 生化免疫特征
AIH-PBC组ALT、AST、TBIL、ALB、GLB水平多介于PBC-AIH OS组与单纯PBC组之间，其中ALT、AST水平低于PBC-AIH OS组，差异有统计学意义(P<0.05)；IgG、IgM、TBIL、GLB水平高于单纯PBC组，差异有统计学意义(P<0.05)；ALB水平高于PBC-AIH OS组，低于单纯PBC组，差异均有统计学意义(P<0.05)。各组ALP、GGT水平差异无统计学意义(P>0.05，表2)。
2.3 自身抗体特征
AIH-PBC组ASMA、SLA/LP、SSB检出率明显低于PBC-AIH OS组，AMA/M2检出率明显高于PBC-AIH OS组，SSA、Ro-52抗体检出率高于单纯PBC组(P<0.05)。各组ANA、Sp100抗体、gp210抗体、LC-1抗体检出率差异无统计学意义(P>0.05，表3)。
2.4 病理分级分期
157例AIH-PBC、50例PBCAIH OS及184例单纯PBC患者初诊时完成肝组织病理检查，秩和检验分析结果显示，各组肝组织病理纤维化分级和炎症分期分布差异有统计学意义(P<0.05)。PBC-AIH OS组纤维化分级(G)和炎症分期(S)的等级均明显高于AIH-PBC组，AIH-PBC组明显高于单纯PBC组(P<0.05)。FIB-4指数及APRI指数等肝纤维化无创评分模型评分结果显示，PBC-AIH OS组FIB-4、APRI指数明显高于AIH-PBC组，且AIH-PBC组明显高于单纯PBC组(P<0.05，图1)。
2.5 预后分析
统计各组初诊未发生不良事件的患者随访5年后不良事件的发生情况，利用Kaplan-Meier生存分析法进行预后比较，结果显示，共纳入PBCAIH OS患者25例，AIH-PBC患者73例，单纯PBC患者85例，三组患者5年累积不良事件发生率分别为52.0%、31.5%、16.5%。Log-rank检验结果显示，PBCAIH OS患者5年累积不良事件发生率明显高于AIHPBC患者(χ2=4.027，P=0.045)，AIH-PBC患者明显高于单纯PBC患者(χ2=4.692，P=0.037，图2)。
3 讨论
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PBC-AIH OS并非PBC与AIH的简单重叠，而是具有其复杂性与异质性。巴黎标准作为EASL推荐的诊断方法，虽较为严格，可避免PBC-AIH OS的过度诊断，但同时可能漏诊部分患者。单纯PBC患者常采用UDCA单药治疗，而PBC-AIH OS患者则常需UDCA联合免疫抑制治疗才可获得生化应答。临床上部分PBC患者伴有AIH特征，但未达到巴黎标准，其诊断介于单纯PBC与PBC-AIH OS之间，称之为伴AIH特征的PBC(AIH-PBC)。目前有关AIH-PBC的研究很少报道，本研究回顾性分析AIH-PBC患者的临床特征。
本研究发现，PBC-AIH OS、AIH-PBC和单纯PBC患者年龄、性别无显著差异，且均以中年女性为主，与自身免疫性肝病(autoimmune liver diseases，AILD)的流行病学研究结果类似。在临床症状上，AILD患者症状通常不具特异性，常无明显症状或因乏力、纳差、瘙痒、黄疸等症状就诊。PBC的疾病发展通常分为临床前期、无症状期、症状期、失代偿期4个阶段[14]。临床前期患者检测仅AMA阳性，生化指标无明显异常；无症状期患者表现为ALP、GGT等胆汁淤积指标异常但无明显临床症状；当出现乏力、瘙痒等症状时则表示已经进入症状期。该疾病发展过程较缓慢，从临床前期发展至症状期通常需要10余年时间，但在此期间肝内中小胆管的破坏却持续进行，这也是部分患者出现临床症状就诊时即发生肝硬化的原因[15]。本研究发现，乏力、瘙痒、口干眼干是三组患者最为常见的症状，但组间差异无统计学意义。在黄疸及纳差方面，AIH-PBC与单纯PBC表现类似，二者的发生率均低于PBC-AIH OS，这一表现可能与PBC-AIH OS患者疾病进展更为迅速有关，这也与先前的报道结果类似[8]。由此可见，若仅通过患者的一般特征及临床症状，很难将三者鉴别。
从生化及免疫指标来看，单纯PBC以胆汁淤积表现为主，表现为反映胆汁淤积的指标上升，如ALP、GGT。ALP广泛分布于人体各组织器官，以肝脏中含量最为丰富，其次为骨骼、肾脏、肠管、胎盘等部位[16]。除儿童生理性增高外，ALP明显升高多与胆汁淤积性肝病、原发或继发性肝细胞癌等疾病相关。正常人血清中的GGT主要来源于肝脏，其在肝内主要分布于肝细胞质及肝内胆管上皮中。GGT通常在急性肝炎、慢性活动性肝炎及失代偿期肝硬化时轻中度升高。而发生胆汁淤积性肝病时，GGT常因排泄不畅逆流入血，可显著升高[17]。AIH的典型生化表现为转氨酶和IgG升高，部分AIH患者的转氨酶高于正常值上限10倍以上，PBC-AIH OS则兼有肝细胞炎症表现，AIH-PBC介于二者之间，但表现更倾向于PBC-AIH OS。PBCAIH OS的ALT、AST水平显著高于AIH-PBC，主要原因可能是利用巴黎标准诊断PBC-AIH OS时，由于患者人群ASMA阳性率较低，通常采用ALT或AST>5×ULN的标准。此外，PBC-AIH OS界面性肝炎表现也较AIH-PBC更常见、更严重，预示肝细胞坏死更重，也可导致转氨酶升高。AIH-PBC的TBIL、IgG、IgM、GLB水平显著高于单纯PBC患者，ALB水平低于单纯PBC患者，表明相较单纯PBC，AIH-PBC免疫反应程度更重，肝细胞合成功能受损也更为明显。有研究发现，IgM与IgG比值可用于鉴别PBC患者是否重叠AIH[18]，提示医师需要关注IgG显著升高的PBC患者是否合并AIH。
本研究自身抗体检测结果显示，AIH-PBC组和单纯PBC组SMA、SLA/LP抗体检出率明显低于PBC-AIH OS组，提示SMA、SLA/LP抗体主要用于PBC-AIH OS与其他AILD的鉴别。而ANA、AMA、Sp100抗体、gp210抗体检出率三组间无显著差异，提示其主要用于PBC的诊断及与AIH的鉴别诊断。本研究中，AIH-PBC和PBC-AIH OS的抗Ro-52抗体检出率明显高于单纯PBC组，这与Saito等[19]的报道相符。值得关注的是，PBC-AIH OS组及AIH-PBC组SSA、SSB抗体的检出率明显高于单纯PBC组，这也显示出AILD重叠状态的复杂性。SSA的抗原包含Ro-52和Ro-60两种蛋白，SSB的抗原为48 kD的磷酸化蛋白[20]。通常认为SSA和SSB与干燥综合征、系统性红斑狼疮等自身免疫性疾病密切相关[21]。流行病学研究发现，AILD患者常同时伴有一种或多种肝外自身免疫性疾病。PBC患者中，40%~60%可检测到干燥综合征相关抗体，5.6%~23.6%可伴有自身免疫性甲状腺炎(autoimmune thyroiditis，AT)，1.4%~12.3%可伴有系统性硬化(systemic sclerosis，SSc)[22]。此外，类风湿关节炎(rheumatoid arthritis，RA)、系统性红斑狼疮、白癜风在AILD患者中亦不少见，增加了临床诊断的难度，原因在于这些肝外自身免疫性疾病可同时伴有肝细胞或胆管上皮的受累。因此在AILD的临床诊治工作中，需要考虑患者是否可能同时伴有其他肝外自身免疫性疾病，以便根据疾病的轻重缓急，制定综合治疗方案。
To等[3]发现，相比一般PBC患者，PBC-AIH OS患者疾病进展更迅速，发生肝硬化、门脉高压、食管静脉曲张、消化道出血、腹水、肝移植及死亡的风险更高。本研究病理及超声检查结果显示，AIHPBC在疾病进展方面与PBC-AIH OS类似，PBC-AIH OS及AIH-PBC初诊患者病理分级分期以及肝纤维化无创评分均显著高于单纯PBC患者，5年累积不良事件发生率高于单纯PBC患者，表明PBC-AIH OS及AIH-PBC患者预后较单纯PBC患者差。因此，加深对AIH-PBC的认识，早期诊断及有效诊治对提高患者生存质量和改善预后尤为重要。
综上所述，AIH-PBC在生化、免疫、病理特征及长期预后方面都与单纯PBC有所不同。在PBC的诊疗过程中应首先甄别出合并干燥综合征或其他自身免疫性疾病的患者，可考虑UDCA联合激素或其他相应方法治疗。其次筛选出符合巴黎标准的患者，并给予UDCA联合激素治疗。对不符合巴黎标准的PBC患者可根据IAIHG简化积分标准界定其是否伴有AIH特征，若简化积分<6分，则评定为单纯PBC并给予UDCA单药治疗，应答不佳时可加用贝特类、吗替麦考酚酯等二线药物；若简化积分≥6分，则评定为AIH-PBC，因其长期预后较单纯PBC差，随访期间应密切观察患者疗效及病情变化，可考虑在UDCA治疗的基础上加用免疫抑制治疗。
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  • 国家自然科学基金(81770569)
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2021年第46卷第1期
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doi: 10.11855/j.issn.0577-7402.2021.01.07
  • 接收时间：2020-06-03
  • 首发时间：2025-12-26
  • 出版时间：2021-01-28
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  • 收稿日期：2020-06-03
  • 修回日期：2020-12-02
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National Natrual Science Foundation of China(81770569)
国家自然科学基金(81770569)
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    空军军医大学附属西京医院消化内科，西安 710032

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鹅膏菌科Amanitaceae 2 11 5.26 鹅膏菌属 Amanita 10 4.78
小菇科 Mycenaceae 2 12 5.74 丝盖伞属 Inocybe 5 2.39
多孔菌科 Polyporaceae 8 14 6.70 蜡蘑属 Laccaria 5 2.39
红菇科 Russulaceae 3 23 11.00 小皮伞属 Marasmius 6 2.87
小菇属 Mycena 11 5.26
光柄菇属 Pluteus 5 2.39
红菇属 Russula 17 8.13
栓菌属 Trametes 5 2.39
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