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Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long‐term outcomes

Background Concurrent extrahepatic autoimmune disease (CEHAID) associated with autoimmune hepatitis (AIH) have been incorporated into the diagnostic criteria stipulated by the International Autoimmune Hepatitis Group (IAIHG). Large comprehensive cohort data on the extrahepatic autoimmunity in AIH re...

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Published in:Liver international 2017-03, Vol.37 (3), p.449-457
Main Authors: Wong, Guan‐Wee, Yeong, Tian, Lawrence, David, Yeoman, Andrew D., Verma, Sumita, Heneghan, Michael A.
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creator Wong, Guan‐Wee
Yeong, Tian
Lawrence, David
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description Background Concurrent extrahepatic autoimmune disease (CEHAID) associated with autoimmune hepatitis (AIH) have been incorporated into the diagnostic criteria stipulated by the International Autoimmune Hepatitis Group (IAIHG). Large comprehensive cohort data on the extrahepatic autoimmunity in AIH remain scanty. Aim To systematically assess features and clinical impact of CEHAID on AIH. Methods Clinical records of 562 patients with AIH from two tertiary centres in the UK were retrospectively reviewed. Results Prevalence of CEHAID in patients with AIH was 42%. Autoimmune thyroid disease was the commonest CEHAID associated with AIH (101/562, 18%). Autoimmune skin diseases were more prevalent in AIH‐2 than AIH‐1 (21.9% vs 7%, P=.009). Personal history of CEHAID was more commonly found in AIH patients with than without first‐degree family history of CEHAID [48/86 (55.8%) vs 169/446 (37.9%), P=.002]. AIH patients with CEHAID were more often women (85.2% vs 76.1%, P=.008), had higher post‐treatment IAIHG score (22 vs 20, P
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Large comprehensive cohort data on the extrahepatic autoimmunity in AIH remain scanty. Aim To systematically assess features and clinical impact of CEHAID on AIH. Methods Clinical records of 562 patients with AIH from two tertiary centres in the UK were retrospectively reviewed. Results Prevalence of CEHAID in patients with AIH was 42%. Autoimmune thyroid disease was the commonest CEHAID associated with AIH (101/562, 18%). Autoimmune skin diseases were more prevalent in AIH‐2 than AIH‐1 (21.9% vs 7%, P=.009). Personal history of CEHAID was more commonly found in AIH patients with than without first‐degree family history of CEHAID [48/86 (55.8%) vs 169/446 (37.9%), P=.002]. AIH patients with CEHAID were more often women (85.2% vs 76.1%, P=.008), had higher post‐treatment IAIHG score (22 vs 20, P&lt;.001), less reactivity to smooth muscle antibodies (49.8% vs 65%, P&lt;.001), more likely to have mild fibrosis at diagnosis (20.9% vs 6.5%, P&lt;.001) and less often had ascites (6.3% vs 13.6%, P=.008) and coagulopathy (1.18 vs 1.27, P=.013) at presentation. Presence of CEHAID, however, did not significantly affect disease progression, prognosis and survival in AIH. Conclusions Our study confirms the strong association of CEHAID with AIH. Association between personal and familial extrahepatic autoimmunity especially among first‐degree relatives was evident. Presence of CEHAID may influence clinical phenotype of AIH at presentation, but without notable impact on the long‐term clinical outcomes.</description><identifier>ISSN: 1478-3223</identifier><identifier>EISSN: 1478-3231</identifier><identifier>DOI: 10.1111/liv.13236</identifier><identifier>PMID: 27541063</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antibodies ; Ascites ; Autoantibodies - blood ; Autoimmune Diseases - classification ; Autoimmune Diseases - epidemiology ; autoimmune skin disease ; autoimmune thyroiditis ; Child ; Child, Preschool ; Databases, Factual ; Diagnosis ; Diagnostic systems ; Disease ; Disease Progression ; extrahepatic autoimmune diseases ; Family Health ; family history ; Female ; Fibrosis ; first‐degree relatives ; Genetics ; Hepatitis ; Hepatitis, Autoimmune - complications ; Humans ; Liver ; Male ; Medical prognosis ; Middle Aged ; Patients ; Prognosis ; Retrospective Studies ; Risk Factors ; Skin diseases ; Smooth muscle ; Survival Analysis ; Tertiary Care Centers ; Thyroid ; United Kingdom - epidemiology ; Young Adult</subject><ispartof>Liver international, 2017-03, Vol.37 (3), p.449-457</ispartof><rights>2016 John Wiley &amp; Sons A/S. 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Published by John Wiley &amp; Sons Ltd.</rights><rights>2017 John Wiley &amp; Sons A/S</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3886-96337c92eb143d6204cd0112676e65f6cc8145ab1c8749be1c556c4135e6be683</citedby><cites>FETCH-LOGICAL-c3886-96337c92eb143d6204cd0112676e65f6cc8145ab1c8749be1c556c4135e6be683</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27541063$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Wong, Guan‐Wee</creatorcontrib><creatorcontrib>Yeong, Tian</creatorcontrib><creatorcontrib>Lawrence, David</creatorcontrib><creatorcontrib>Yeoman, Andrew D.</creatorcontrib><creatorcontrib>Verma, Sumita</creatorcontrib><creatorcontrib>Heneghan, Michael A.</creatorcontrib><title>Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long‐term outcomes</title><title>Liver international</title><addtitle>Liver Int</addtitle><description>Background Concurrent extrahepatic autoimmune disease (CEHAID) associated with autoimmune hepatitis (AIH) have been incorporated into the diagnostic criteria stipulated by the International Autoimmune Hepatitis Group (IAIHG). Large comprehensive cohort data on the extrahepatic autoimmunity in AIH remain scanty. Aim To systematically assess features and clinical impact of CEHAID on AIH. Methods Clinical records of 562 patients with AIH from two tertiary centres in the UK were retrospectively reviewed. Results Prevalence of CEHAID in patients with AIH was 42%. Autoimmune thyroid disease was the commonest CEHAID associated with AIH (101/562, 18%). Autoimmune skin diseases were more prevalent in AIH‐2 than AIH‐1 (21.9% vs 7%, P=.009). Personal history of CEHAID was more commonly found in AIH patients with than without first‐degree family history of CEHAID [48/86 (55.8%) vs 169/446 (37.9%), P=.002]. AIH patients with CEHAID were more often women (85.2% vs 76.1%, P=.008), had higher post‐treatment IAIHG score (22 vs 20, P&lt;.001), less reactivity to smooth muscle antibodies (49.8% vs 65%, P&lt;.001), more likely to have mild fibrosis at diagnosis (20.9% vs 6.5%, P&lt;.001) and less often had ascites (6.3% vs 13.6%, P=.008) and coagulopathy (1.18 vs 1.27, P=.013) at presentation. Presence of CEHAID, however, did not significantly affect disease progression, prognosis and survival in AIH. Conclusions Our study confirms the strong association of CEHAID with AIH. Association between personal and familial extrahepatic autoimmunity especially among first‐degree relatives was evident. Presence of CEHAID may influence clinical phenotype of AIH at presentation, but without notable impact on the long‐term clinical outcomes.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Antibodies</subject><subject>Ascites</subject><subject>Autoantibodies - blood</subject><subject>Autoimmune Diseases - classification</subject><subject>Autoimmune Diseases - epidemiology</subject><subject>autoimmune skin disease</subject><subject>autoimmune thyroiditis</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Databases, Factual</subject><subject>Diagnosis</subject><subject>Diagnostic systems</subject><subject>Disease</subject><subject>Disease Progression</subject><subject>extrahepatic autoimmune diseases</subject><subject>Family Health</subject><subject>family history</subject><subject>Female</subject><subject>Fibrosis</subject><subject>first‐degree relatives</subject><subject>Genetics</subject><subject>Hepatitis</subject><subject>Hepatitis, Autoimmune - complications</subject><subject>Humans</subject><subject>Liver</subject><subject>Male</subject><subject>Medical prognosis</subject><subject>Middle Aged</subject><subject>Patients</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><subject>Risk Factors</subject><subject>Skin diseases</subject><subject>Smooth muscle</subject><subject>Survival Analysis</subject><subject>Tertiary Care Centers</subject><subject>Thyroid</subject><subject>United Kingdom - epidemiology</subject><subject>Young Adult</subject><issn>1478-3223</issn><issn>1478-3231</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><recordid>eNp1kcuKFTEQhoMozkUXvoAE3MyAZ6Yrt07Pbjh4GTjgRt2GdLrOmKE7OSZp9ezEJ_AZfRKjPY4gWJuqn_r4qeIn5Ak0Z1DrfPSfzoAzru6RQxCtXtUZ7t_NjB-Qo5xvmga6TsJDcsBaKaBR_JB8W8fg5pQwFIpfSrIfcGeLd9TOJfppmoMve-rDX410QYrPF9RPu9G7qmLIdBsTHby9DjH7_Jy60Ye6G6mLc8pIbRjoGMP1j6_fC6aJxrm4OGF-RB5s7Zjx8W0_Ju9evni7fr3avHl1tb7crBzXWq06xXnrOoY9CD4o1gg3NABMtQqV3CrnNAhpe3C6FV2P4KRUTgCXqHpUmh-Tk8V3l-LHGXMxk88Ox9EGjHM2oGXXMs60qOizf9Cb-kOo1xnoWCOZlqKr1OlCuRRzTrg1u-Qnm_YGGvMrGFODMb-DqezTW8e5n3C4I_8kUYHzBfjsR9z_38lsrt4vlj8BvQaaWg</recordid><startdate>201703</startdate><enddate>201703</enddate><creator>Wong, Guan‐Wee</creator><creator>Yeong, Tian</creator><creator>Lawrence, David</creator><creator>Yeoman, Andrew D.</creator><creator>Verma, Sumita</creator><creator>Heneghan, Michael A.</creator><general>Wiley Subscription Services, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7QO</scope><scope>7T5</scope><scope>7U9</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope></search><sort><creationdate>201703</creationdate><title>Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long‐term outcomes</title><author>Wong, Guan‐Wee ; 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Large comprehensive cohort data on the extrahepatic autoimmunity in AIH remain scanty. Aim To systematically assess features and clinical impact of CEHAID on AIH. Methods Clinical records of 562 patients with AIH from two tertiary centres in the UK were retrospectively reviewed. Results Prevalence of CEHAID in patients with AIH was 42%. Autoimmune thyroid disease was the commonest CEHAID associated with AIH (101/562, 18%). Autoimmune skin diseases were more prevalent in AIH‐2 than AIH‐1 (21.9% vs 7%, P=.009). Personal history of CEHAID was more commonly found in AIH patients with than without first‐degree family history of CEHAID [48/86 (55.8%) vs 169/446 (37.9%), P=.002]. AIH patients with CEHAID were more often women (85.2% vs 76.1%, P=.008), had higher post‐treatment IAIHG score (22 vs 20, P&lt;.001), less reactivity to smooth muscle antibodies (49.8% vs 65%, P&lt;.001), more likely to have mild fibrosis at diagnosis (20.9% vs 6.5%, P&lt;.001) and less often had ascites (6.3% vs 13.6%, P=.008) and coagulopathy (1.18 vs 1.27, P=.013) at presentation. Presence of CEHAID, however, did not significantly affect disease progression, prognosis and survival in AIH. Conclusions Our study confirms the strong association of CEHAID with AIH. Association between personal and familial extrahepatic autoimmunity especially among first‐degree relatives was evident. Presence of CEHAID may influence clinical phenotype of AIH at presentation, but without notable impact on the long‐term clinical outcomes.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>27541063</pmid><doi>10.1111/liv.13236</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record>
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source Wiley:Jisc Collections:Wiley Read and Publish Open Access 2024-2025 (reading list)
subjects Adolescent
Adult
Aged
Aged, 80 and over
Antibodies
Ascites
Autoantibodies - blood
Autoimmune Diseases - classification
Autoimmune Diseases - epidemiology
autoimmune skin disease
autoimmune thyroiditis
Child
Child, Preschool
Databases, Factual
Diagnosis
Diagnostic systems
Disease
Disease Progression
extrahepatic autoimmune diseases
Family Health
family history
Female
Fibrosis
first‐degree relatives
Genetics
Hepatitis
Hepatitis, Autoimmune - complications
Humans
Liver
Male
Medical prognosis
Middle Aged
Patients
Prognosis
Retrospective Studies
Risk Factors
Skin diseases
Smooth muscle
Survival Analysis
Tertiary Care Centers
Thyroid
United Kingdom - epidemiology
Young Adult
title Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long‐term outcomes
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