Autoimmune Liver Disease

John M. Vierling MD, FACP, FAASLD, AGAFProfessor of Medicine and Surgery, Chief of Hepatology, Director of Baylor Liver Health, Director of Advanced Liver Therapies, Program Director, Hepatology and Liver Transplantation Fellowship Division of Abdominal Transplantation, Michael E. DeBakey Department of Surgery, Section of Gastroenterology and Hepatology, Margaret M. and Albert B. Alkek Department of Medicine, Baylor College of Medicine, Houston, Texas, USA

Ewa Wunsch MDTranslational Medicine Group, Pomeranian Medical University, Szczecin, Poland

AASLDAmerican Association for the Study of Liver DiseasesABCATP‐binding cassetteABDautoimmune biliary diseaseACAanti‐centromere antibodyACRAmerican College of RheumatologyADCCantibody‐dependent cell‐mediated cytotoxicityAFLPacute fatty liver disease of pregnancyAIHautoimmune hepatitisAILDautoimmune liver diseaseAIPautoimmune pancreatitisAIREautoimmune regulatorAISCautoimmune sclerosing cholangitisALFacute liver failureALPalkaline phosphataseALTalanine aminotransferaseAMAanti‐mitochondrial antibodyANAanti‐nuclear antibodyANCAanti‐neutrophil cytoplasmic antibodyAPCantigen‐presenting cellAPECEDautoimmune polyendocrinopathy/candidiasis/ectodermal dystrophyAPRIAST‐to‐platelet ratio indexAPS‐1autoimmune polyendocrine syndrome type 1ASCautoimmune sclerosing cholangitisASGPRasialoglycoprotein receptorASMAanti‐smooth muscle antibodyASTaspartate aminotransferaseATXautotaxinBAbile acidBCOADCbranched‐chain 2‐oxo acid dehydrogenase complexBCRB‐cell receptorBECbiliary epithelial cellBETbromodomain and extra‐terminalBLCB‐lymphocyte chemoattractantBMDbone mineral densityBregB regulatory cellBSEPbile salt export pumpBTSCbiliary tree stem/progenitor cellC′complementCA19‐9carbohydrate antigen 19‐9CCcryptogenic cirrhosisCCHcryptogenic chronic hepatitisCDceliac diseaseCDCAchenodeoxycholic acidCIconfidence intervalCNSDCchronic non‐suppurative destructive cholangitisCREBcAMP response element binding proteinCSCesarean sectionCTcomputed tomographyCTDconnective tissue diseaseCTLcytotoxic T lymphocyteCTLAcytotoxic T‐lymphocyte antigenCYPcytochrome PD31,25‐dihydroxyvitamin D3DAGdiacylglycerolDAMPdamage‐associated molecular patternDCdendritic cellDCDdonation after circulatory deathDCregregulatory dendritic cellDDLTdeceased donor liver transplantDILIdrug‐induced liver injuryDOCK1dedicator of cytokinesis 1 (protein)DXAdual‐energy X‐ray absorptiometryEASLEuropean Association for the Study of the LiverEGDesophagogastroduodenoscopyEHAIDextrahepatic autoimmune diseaseELFenhanced liver fibrosis (test)ELISAenzyme‐linked immunosorbent assayELTREuropean Liver Transplant RegistryENAextractable nuclear antigeneQTLexpression quantitative trait locusERCPendoscopic retrograde cholangiopancreatographyeRNAenhancer RNAEULAREuropean League Against RheumatismEUSendoscopic ultrasoundEVLendoscopic variceal ligationFDAFood and Drug AdministrationFISHfluorescence in situ hybridizationFNAfine needle aspirationFoxPforkhead box P3FXRfarnesoid X receptorGEFguanine nucleotide exchange factorGGTgamma‐glutamyltranspeptidase (or gamma‐glutamyltransferase)GLPglucagon‐like peptideGWASgenome‐wide association studiesHAIhepatic/hepatitis activity indexHBRVhuman betaretrovirusHBVhepatitis B virusHCChepatocellular carcinomaHCVhepatitis C virusHDLhigh‐density lipoproteinHLAhuman leukocyte antigenHMG‐CoA3‐hydroxy‐3‐methylglutaryl‐coenzyme AHPFhigh‐power fieldHpSChepatic stem/progenitor cellHRhazard ratioHRQoLhealth‐related quality of lifeHSChepatic stellate cellHSEChepatic sinusoidal endothelial cellHSVherpes simplex virusIACIgG4‐associated cholangitisIAHInternational Autoimmune Hepatitis (score)IAIHGInternational IAH GroupIBDinflammatory bowel diseaseICUintensive care unitIgG4‐RDIgG4‐related diseaseIIFindirect immunofluorescenceILinterleukinINRinternational normalized ratioIPEXimmunodysregulation polyendocrinopathy enteropathy X‐linked (syndrome)IRFinterferon regulatory factorISREinterferon‐stimulated response elementJAKJanus kinaseLAKlymphokine‐activated killer (cell)LC1liver cytosol type 1 (antibody)LDlinkage disequilibriumLDLlow‐density lipoproteinLDLTliving donor liver transplantLElupus erythematosusLFTliver function testLKM1liver kidney microsomal type 1 (antibody)lncRNAlong non‐coding RNALPAlysophosphatidic acidLPSlipopolysaccharideLSECliver sinusoidal endothelial cellLTliver transplantationMAGUKmembrane‐associated guanylate kinaseMAITmucosal invariant T (cell)MAPKmitogen‐activated protein kinaseMDRmultidrug resistanceMELDModel for End‐stage Liver Disease (score)MHCmajor histocompatibility complexMIFmacrophage migration inhibitory factormiRNAmicroRNAMMFmycophenolate mofetil6‐MMP6‐methylmercaptopurineMMTVmouse mammary tumor virus6‐MP6‐mercaptopurinemQTLmethylation quantitative trait locusMRCmagnetic resonance cholangiographyMRCPmagnetic resonance cholangiopancreatographyMRPmultidrug resistance proteinMSmultiple sclerosismTECmedullary thymic epithelial cellNACN‐acetylcysteineNAFLDnon‐alcoholic fatty liver diseaseNANAnuclear anti‐neutrophil antibodyNASHnon‐alcoholic steatohepatitisNETneutrophil extracellular trapNFκBnuclear factor kappa BNICENational Institute for Health and Care Excellence (UK)NICUneonatal intensive care unitNKnatural killer (cell)NKTnatural killer T (cell)NLRP3nucleotide‐binding oligomerization domain (NOD)‐like receptor P3NODnon‐obese diabeticNSAIDnon‐steroidal anti‐inflammatory drugNTCPsodium taurocholate cotransporting polypeptideOATPorganic anion‐transporting polypeptideOCAobeticholic acidOGDC2‐oxoglutarate dehydrogenase complexOPGosteoprotegerinORodds ratioOSoverlap syndromePAMPpathogen‐associated molecular patternpANCAperinuclear anti‐neutrophil cytoplasmic antibodypANNAperinuclear anti‐neutrophil nuclear antibodyPBCprimary biliary cholangitisPBGperibiliary glandPD‐1programmed cell death 1PDCpyruvate dehydrogenase complexPETpositron emission tomographyPKCprotein kinase CP1NPprocollagen type 1 N‐terminal propeptidePPARperoxisome proliferator‐activated receptorPRRpattern recognition receptorPSCprimary sclerosing cholangitisPXRpregnane X receptorQALYquality‐adjusted life‐yearRArheumatoid arthritisRAGrecombination activating generAIHrecurrent autoimmune hepatitisRANKLreceptor activator of nuclear factor kappa‐B ligandRDCreactive ductular cellRFrheumatoid factorRORreceptor‐related orphan receptorrPBCrecurrent primary biliary cholangitisrPSCrecurrent primary sclerosing cholangitisRRrelative riskRTKreceptor tyrosine kinaseRXRretinoid X receptorSASPsenescence‐associated secretory phenotypeSCIDsevere combined immunodeficiencySEsuper enhancerSH2BSH2B adapter protein 3SLAsoluble liver antigenSLEsystemic lupus erythematosusSMAsmooth muscle antibodySNPsingle nucleotide polymorphismSSSjögren syndromeSScsystemic sclerosisSTATsignal transducer and activatorTCRT‐cell receptorT1DMtype 1 diabetes mellitusTFtranscription factorTfhT follicular helper (cell)TGFtransforming growth factor6‐TGN6‐thioguanine nucleotideTIPStransjugular intrahepatic portosystemic shuntTLRToll‐like receptorTLStertiary lymphoid structureTNFtumor necrosis factorTNFAIP3TNF‐induced protein 3TNIP1TNFα‐induced protein 3‐interacting protein 1TORCtransducer of regulated CREB activityTPMTthiopurine S‐methyltransferaseTRAILTNF‐apoptosis‐inducing ligandTregT regulatory cellTRMtissue‐resident memory T (cell)UCulcerative colitisUDCAursodeoxycholic acidUGTuridine diphosphate glucuronosyltransferaseULNupper limit of normalUTIurinary tract infectionVBvariceal bleedingVDRvitamin D receptorWDWilson disease

M. Eric Gershwin

Division of Rheumatology, Allergy and Clinical Immunology, University of California School of Medicine, Davis, CA, USA