Overlap of concurrent extrahepatic autoimmune diseases is associated with milder disease severity of newly diagnosed autoimmune hepatitis

作     者：Tobias Mühling Helmut Rohrbach Wolfgang Schepp Felix Gundling Tobias Mühling;Helmut Rohrbach;Wolfgang Schepp;Felix Gundling

作者机构：Department of GastroenterologyInternal Medicine ⅡUniversity of WürzburgWürzburgGermany Department of PathologyAcademic Teaching Hospital BogenhausenTechnical University of MunichMunich 81925Germany Department of GastroenterologyHepatology and Gastrointestinal OncologyAcademic Teaching Hospital BogenhausenTechnical University of MunichMunich 81925Germany Department of Internal MedicineDivision of GastroenterologyGastrointestinal Oncology and DiabetologyKemperhof KoblenzKoblenzGermany 

出 版 物：《Hepatobiliary & Pancreatic Diseases International》 (国际肝胆胰疾病杂志（英文版）)

年 卷 期：2021年第20卷第1期

页      面：21-27页

核心收录：

学科分类：1002[医学-临床医学] 10[医学] 

主　　题：Autoimmune hepatitis Concurrent extrahepatic autoimmune disease Serum aminotransferase activities 

摘      要：Background:Concurrent extrahepatic autoimmune disorders(CEHAID)are frequently observed in autoimmune hepatitis(AIH).It is not clear whether there is any prognostic significance of CEHAID on AIH.The aim of this study was to examine the prognostic impact of CEHAID and the correlation with the disease severity of AIH.Methods:This study included 65 hospitalized subjects who fulfilled the accepted criteria for AIH during an 8-year period(2009–2016).All records were manually screened for presence of associated autoimmune diseases.Disease severity of AIH was assessed by liver laboratory tests including the ratio of aspartate aminotransferase to alanine aminotransferase(AST/ALT)and liver histology.Results:Among the enrolled patients,52(80%)were female(median age 61 years,IQR 45–75).Fifty-six(86.2%)were classified as type-1 AIH.In 26(40%)patients at least one additional extrahepatic autoimmune disease was diagnosed.Thirty-four subjects were referred to our hospital because of acute presentation of AIH(supposed by an acute elevation of hepatic enzymes)for subsequent liver biopsy resulting in initial diagnosis of AIH.This group was stratified into 3 subgroups:(A)AIH alone(n=14);(B)overlap with primary biliary cirrhosis(PBC)/primary sclerosing cholangitis(PSC)(n=11);and(C)with CEHAID(n=9).AST/ALT ratio was the lowest in subgroup C(median 0.64,IQR 0.51–0.94;P=0.023),compared to subgroup A(median 0.91,IQR 0.66–1.10)and subgroup B(median 1.10,IQR 0.89–1.36).Patients with AIH alone showed a trend to the highest grade of fibrosis(mean 2.3;95%CI:1.5–3.0)with no statistical significance compared to subjects with CEHAID(lowest grade of fibrosis;mean 1.5;95%CI:0.2–2.8;P=0.380)whereas the ongoing inflammation was comparable.Conclusions:AST/ALT ratio and extent of fibrosis were lower in subjects with AIH and CEHAID,compared to subjects with only AIH.Therefore,the occurrence of CEHAID might be a predictor for lower disease severity of newly diagnosed acute onset AIH,possibly caused by an earlier diagnosis or different modes of damage.