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Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long term outcome
journal contribution
posted on 2023-06-09, 02:53 authored by Guan-Wee Wong, Tian Yeong, David Lawrence, Andrew D Yeoman, Sumita VermaSumita Verma, Michael A HeneghanConcurrent 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 United Kingdom were retrospectively reviewed RESULTS: Prevalence of CEHAID in patients with AIH were 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=0.009). 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=0.002]. AIH patients with CEHAID were more often female [201/236 (85.2%), p=0.008], had higher post-treatment IAIHG score (22 vs. 20, p<0.001), less reactivity to smooth muscle antibodies (49.8% vs 65%, p<0.001), more likely to have mild fibrosis at diagnosis (20.9% vs. 6.5%, p<0.001), less often had ascites (6.3% vs. 13.6%, p=0.008) and coagulopathy (1.18 vs. 1.27, p=0.013) 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
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Publication status
- Published
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- Accepted version
Journal
Liver InternationalISSN
1478-3223Publisher
WileyExternal DOI
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3Volume
37Page range
449-457Department affiliated with
- Clinical and Experimental Medicine Publications
Full text available
- Yes
Peer reviewed?
- Yes
Legacy Posted Date
2016-09-14First Open Access (FOA) Date
2017-09-24First Compliant Deposit (FCD) Date
2016-09-14Usage metrics
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