{"id":"https://openalex.org/W2744820174","doi":null,"title":"The immunopathology of primary biliary cirrhosis: thoughts for the millennium.","display_name":"The immunopathology of primary biliary cirrhosis: thoughts for the millennium.","publication_year":2000,"publication_date":"2000-01-01","ids":{"openalex":"https://openalex.org/W2744820174","mag":"2744820174","pmid":"https://pubmed.ncbi.nlm.nih.gov/10722225"},"language":"en","primary_location":{"id":"pmid:10722225","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/10722225","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Archivum immunologiae et therapiae experimentalis","raw_type":"Journal Article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5064494964","display_name":"Motoko Sasaki","orcid":"https://orcid.org/0000-0003-2514-760X","observed_orcids":["https://orcid.org/0000-0003-2514-760X"]},"institutions":[{"id":"https://openalex.org/I84218800","display_name":"University of California, Davis","ror":"https://ror.org/05rrcem69","country_code":"US","type":"education","lineage":["https://openalex.org/I84218800"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"M Sasaki","raw_affiliation_strings":["Division of Allergy, Rheumatology, and Clinical Immunology, University of California, Davis, School of Medicine, 95616, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Allergy, Rheumatology, and Clinical Immunology, University of California, Davis, School of Medicine, 95616, USA","institution_ids":["https://openalex.org/I84218800"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5102964668","display_name":"Aftab Ahmed Ansari","orcid":"https://orcid.org/0000-0003-4607-7268","observed_orcids":["https://orcid.org/0000-0003-4607-7268"]},"institutions":[{"id":"https://openalex.org/I150468666","display_name":"Emory University","ror":"https://ror.org/03czfpz43","country_code":"US","type":"education","lineage":["https://openalex.org/I150468666"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"A A Ansari","raw_affiliation_strings":["Emory University"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Emory University","institution_ids":["https://openalex.org/I150468666"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5008003570","display_name":"Yasuni Nakanuma","orcid":"https://orcid.org/0000-0003-4117-6611","observed_orcids":["https://orcid.org/0000-0003-4117-6611"]},"institutions":[{"id":"https://openalex.org/I10091056","display_name":"Kanazawa University","ror":"https://ror.org/02hwp6a56","country_code":"JP","type":"education","lineage":["https://openalex.org/I10091056"]}],"countries":["JP"],"is_corresponding":false,"raw_author_name":"Y Nakanuma","raw_affiliation_strings":["Kanazawa University"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Kanazawa University","institution_ids":["https://openalex.org/I10091056"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5016565634","display_name":"Ross L. Coppel","orcid":"https://orcid.org/0000-0002-4476-9124","observed_orcids":["https://orcid.org/0000-0002-4476-9124"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"R L Coppel","raw_affiliation_strings":["Microbiology"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Microbiology","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5113636460","display_name":"Emmet B. Keeffe","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I97018004","display_name":"Stanford University","ror":"https://ror.org/00f54p054","country_code":"US","type":"education","lineage":["https://openalex.org/I97018004"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"E B Keeffe","raw_affiliation_strings":["Stanford University ()"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Stanford University ()","institution_ids":["https://openalex.org/I97018004"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5110194091","display_name":"M. Eric Gershwin","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"M E Gershwin","raw_affiliation_strings":["Rheumatology, Allergy, and Clinical Immunology"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Rheumatology, Allergy, and Clinical Immunology","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":2,"institutions_distinct_count":4,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":0.4746,"has_fulltext":false,"cited_by_count":7,"citation_normalized_percentile":{"value":0.72444196,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"48","issue":"1","first_page":"1","last_page":"10"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11834","display_name":"Liver Diseases and Immunity","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2721","display_name":"Hepatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T11834","display_name":"Liver Diseases and Immunity","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2721","display_name":"Hepatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11576","display_name":"IgG4-Related and Inflammatory Diseases","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/primary-biliary-cholangitis","display_name":"primary biliary cirrhosis","score":1.0},{"id":"https://openalex.org/keywords/immunopathology","display_name":"immunopathology","score":0.8640000224113464},{"id":"https://openalex.org/keywords/antimitochondrial-antibodies","display_name":"antimitochondrial antibodies","score":0.8730000257492065},{"id":"https://openalex.org/keywords/mitochondrial-autoantigens","display_name":"mitochondrial autoantigens","score":0.9169999957084656},{"id":"https://openalex.org/keywords/autoantibody-epitopes","display_name":"autoantibody epitopes","score":0.8220000267028809},{"id":"https://openalex.org/keywords/t-cell-mediated-cytotoxicity","display_name":"T cell cytotoxicity","score":0.9610000252723694},{"id":"https://openalex.org/keywords/bile-duct-destruction","display_name":"bile duct destruction","score":0.7260000109672546},{"id":"https://openalex.org/keywords/cholestatic-liver-failure","display_name":"cholestatic liver failure","score":0.8399999737739563},{"id":"https://openalex.org/keywords/female-predominance","display_name":"female predominance","score":0.7519999742507935},{"id":"https://openalex.org/keywords/immunosuppressive-therapy","display_name":"immunosuppressive therapy","score":0.7239999771118164},{"id":"https://openalex.org/keywords/liver-fibrosis","display_name":"liver fibrosis","score":0.625},{"id":"https://openalex.org/keywords/portal-inflammation","display_name":"portal inflammation","score":0.6790000200271606}],"concepts":[{"id":"https://openalex.org/C2780366471","wikidata":"https://www.wikidata.org/wiki/Q1072420","display_name":"Primary biliary cirrhosis","level":2,"score":0.9152185916900635},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.6494120955467224},{"id":"https://openalex.org/C163764329","wikidata":"https://www.wikidata.org/wiki/Q785022","display_name":"Autoantibody","level":3,"score":0.5603929162025452},{"id":"https://openalex.org/C195616568","wikidata":"https://www.wikidata.org/wiki/Q128711","display_name":"Epitope","level":3,"score":0.5456777215003967},{"id":"https://openalex.org/C2780648854","wikidata":"https://www.wikidata.org/wiki/Q2745824","display_name":"Immunopathology","level":2,"score":0.48378297686576843},{"id":"https://openalex.org/C2780615123","wikidata":"https://www.wikidata.org/wiki/Q1058608","display_name":"Primary sclerosing cholangitis","level":3,"score":0.4675440192222595},{"id":"https://openalex.org/C2779075594","wikidata":"https://www.wikidata.org/wiki/Q8084905","display_name":"Autoimmune disease","level":3,"score":0.4565860629081726},{"id":"https://openalex.org/C2780788601","wikidata":"https://www.wikidata.org/wiki/Q12233655","display_name":"Intrahepatic bile ducts","level":3,"score":0.4565843939781189},{"id":"https://openalex.org/C147483822","wikidata":"https://www.wikidata.org/wiki/Q103537","display_name":"Antigen","level":2,"score":0.44678768515586853},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.4465767443180084},{"id":"https://openalex.org/C159654299","wikidata":"https://www.wikidata.org/wiki/Q79460","display_name":"Antibody","level":2,"score":0.42522573471069336},{"id":"https://openalex.org/C2780130043","wikidata":"https://www.wikidata.org/wiki/Q192360","display_name":"Autoimmunity","level":3,"score":0.4153348505496979},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.3711671233177185},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.33265221118927},{"id":"https://openalex.org/C2779777945","wikidata":"https://www.wikidata.org/wiki/Q851111","display_name":"Bile duct","level":2,"score":0.2784261405467987},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.2760998606681824},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.09402197599411011}],"mesh":[{"descriptor_ui":"D001323","descriptor_name":"Autoantibodies","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D001324","descriptor_name":"Autoantigens","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006680","descriptor_name":"HLA Antigens","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007249","descriptor_name":"Inflammation","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D007249","descriptor_name":"Inflammation","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D008105","descriptor_name":"Liver Cirrhosis, Biliary","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D008105","descriptor_name":"Liver Cirrhosis, Biliary","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":true},{"descriptor_ui":"D008105","descriptor_name":"Liver Cirrhosis, Biliary","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008928","descriptor_name":"Mitochondria","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D013601","descriptor_name":"T-Lymphocytes","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D015551","descriptor_name":"Autoimmunity","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D015815","descriptor_name":"Cell Adhesion Molecules","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D016207","descriptor_name":"Cytokines","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D016693","descriptor_name":"Receptors, Antigen, T-Cell, alpha-beta","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D017209","descriptor_name":"Apoptosis","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"pmid:10722225","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/10722225","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Archivum immunologiae et therapiae experimentalis","raw_type":"Journal Article"},{"id":"mag:2744820174","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/10722225","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"display_name":"Good health and well-being","score":0.98580002784729,"id":"https://metadata.un.org/sdg/3"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":55,"referenced_works":["https://openalex.org/W1503866127","https://openalex.org/W1513720018","https://openalex.org/W1526505522","https://openalex.org/W1594523905","https://openalex.org/W1646114581","https://openalex.org/W1832430407","https://openalex.org/W1936555387","https://openalex.org/W1968303555","https://openalex.org/W1969961058","https://openalex.org/W1970755428","https://openalex.org/W1974778418","https://openalex.org/W1981964880","https://openalex.org/W1987123144","https://openalex.org/W2006885822","https://openalex.org/W2011393733","https://openalex.org/W2016901949","https://openalex.org/W2019416317","https://openalex.org/W2022097532","https://openalex.org/W2030083654","https://openalex.org/W2037293056","https://openalex.org/W2037832428","https://openalex.org/W2040309484","https://openalex.org/W2040375465","https://openalex.org/W2040844919","https://openalex.org/W2046971569","https://openalex.org/W2051079941","https://openalex.org/W2055420557","https://openalex.org/W2064048801","https://openalex.org/W2078639165","https://openalex.org/W2078797149","https://openalex.org/W2079671748","https://openalex.org/W2080938189","https://openalex.org/W2082702784","https://openalex.org/W2083311645","https://openalex.org/W2086002849","https://openalex.org/W2096139310","https://openalex.org/W2103038330","https://openalex.org/W2106004417","https://openalex.org/W2109620188","https://openalex.org/W2112887558","https://openalex.org/W2117069079","https://openalex.org/W2119967316","https://openalex.org/W2123036265","https://openalex.org/W2132286068","https://openalex.org/W2132632746","https://openalex.org/W2142684897","https://openalex.org/W2148946659","https://openalex.org/W2157379028","https://openalex.org/W2161868454","https://openalex.org/W2396116692","https://openalex.org/W2410185934","https://openalex.org/W2410302322","https://openalex.org/W2414262104","https://openalex.org/W2735656006","https://openalex.org/W4234791281"],"related_works":["https://openalex.org/W2148082560","https://openalex.org/W2617245642","https://openalex.org/W2602740512","https://openalex.org/W1966656092","https://openalex.org/W4232057027","https://openalex.org/W2024373539","https://openalex.org/W1983375163","https://openalex.org/W2050679631","https://openalex.org/W2053637155","https://openalex.org/W2089241323"],"abstract_inverted_index":{"Primary":[0],"biliary":[1,43],"cirrhosis":[2,44],"is":[3,16,46,66,186],"an":[4],"organ":[5],"specific":[6,203],"autoimmune":[7],"disease":[8,19],"that":[9],"produces":[10],"progressive":[11,25],"cholestatic":[12],"liver":[13,63],"failure.":[14],"It":[15],"predominantly":[17],"a":[18],"of":[20,27,41,49,71,79,85,91,114,125,134,147,163,175,183,191,198,205,221],"women":[21],"characterized":[22],"by":[23,55],"chronic":[24],"destruction":[26],"small":[28],"intrahepatic":[29],"bile":[30,105],"ducts":[31],"with":[32,223],"portal":[33],"inflammation":[34],"and":[35,57,88,118,129,141,144,201,216],"ultimately":[36],"fibrosis.":[37],"The":[38,53,132],"serologic":[39],"hallmark":[40],"primary":[42],"(PBC)":[45],"the":[47,69,80,83,89,102,111,122,137,145,160,181,189,195,202,219],"presence":[48,70],"antibodies":[50,61,73,127],"to":[51,139,156],"mitochondria.":[52],"mechanisms":[54,96],"which":[56,59,171],"if":[58],"such":[60],"produce":[62],"tissue":[64],"injury":[65],"unknown.":[67],"However,":[68],"these":[72,214],"have":[74,153],"allowed":[75],"detailed":[76],"immunological":[77],"definition":[78],"antigenic":[81],"epitopes,":[82,143],"nature":[84],"reacting":[86],"autoantibodies":[87],"characterization":[90],"T":[92,115],"cell":[93],"responses.":[94],"Several":[95],"may":[97],"now":[98],"be":[99],"proposed":[100],"regarding":[101],"immune":[103],"mediated":[104],"duct":[106],"damage":[107],"in":[108,148,158,165,193],"PBC,":[109],"including":[110,180],"possible":[112],"role":[113,204],"cell-mediated":[116],"cytotoxicity":[117],"intracellular":[119],"interaction":[120],"between":[121],"IgA":[123],"class":[124],"antimitochondrial":[126],"(AMA)":[128],"mitochondrial":[130,206],"autoantigens.":[131],"advent":[133],"molecular":[135],"biology,":[136],"ability":[138],"clone":[140],"define":[142],"use":[146],"situ":[149],"nucleic":[150],"acid":[151],"hybridization,":[152],"all":[154],"led":[155],"advances":[157],"understanding":[159],"natural":[161],"history":[162],"immunopathology":[164],"PBC.":[166],"There":[167],"are":[168],"major":[169],"questions":[170,182],"remain":[172],"unanswered,":[173],"including,":[174],"course,":[176],"etiology,":[177],"but":[178],"also":[179],"why":[184],"there":[185],"female":[187],"predominance,":[188],"absence":[190],"PBC":[192],"children,":[194],"relative":[196],"ineffectiveness":[197],"immunosuppressive":[199],"drugs,":[200],"antigens.":[207],"In":[208],"this":[209,224],"review,":[210],"we":[211],"focus":[212],"on":[213,218],"issues":[215],"particularly":[217],"immunobiology":[220],"patients":[222],"disease.":[225]},"counts_by_year":[],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2017-08-17T00:00:00"}
