{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T22:43:37Z","timestamp":1784241817742,"version":"3.55.0"},"reference-count":29,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","license":[{"start":{"date-parts":[[2021,4,27]],"date-time":"2021-04-27T00:00:00Z","timestamp":1619481600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2021,4,27]],"date-time":"2021-04-27T00:00:00Z","timestamp":1619481600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Hepatology"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:sec>\n            <jats:title>Background and Aims<\/jats:title>\n            <jats:p>The etiology of biliary atresia (BA) is not known and is likely multifactorial, including a genetic predisposition, a viral or environmental trigger, an aberrant autoimmune response targeting cholangiocytes, and unique susceptibilities of the neonatal bile ducts to injury. Damaged cholangiocytes may express neo self\u2010antigens and elicit autoreactive T\u2010cell\u2010mediated inflammation and B\u2010cell production of autoantibodies. The aim of this study was to discover autoantibodies in BA that correlated with outcomes.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Approach and Results<\/jats:title>\n            <jats:p>An autoantigen microarray encompassing approximately 9,500 autoantigens was used to screen for serum immunoglobulin M (IgM) and immunoglobulin G (IgG) autoantibodies in patients with BA or other liver disease controls. Validation of candidate autoantibodies by enzyme\u2010linked immunosorbent assay on a second cohort of subjects (6\u201012\u00a0months following Kasai portoenterostomy) and correlations of autoantibodies with outcomes were performed (serum bilirubin levels and need for liver transplant in first 2\u00a0years of life). Mean anti\u2010chitinase 3\u2010like 1 (CHI3L1), anti\u2010delta\u2010like ligand (DLL\u20104), and antisurfactant protein D (SFTPD) IgM autoantibodies in BA were significantly higher compared with controls, and IgM autoantibody levels positively correlated with worse outcomes. Immunofluorescence revealed cholangiocyte\u2010predominant expression of CHI3L1, DLL\u20104, and SFTPD. The humoral autoantibody response was associated with C3d complement activation and T\u2010cell autoimmunity, based on detection of cholangiocyte\u2010predominant C3d co\u2010staining and peripheral blood autoreactive T cells specific to CHI3L1, DLL\u20104 and SFTPD, respectively.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>BA is associated with cholangiocyte\u2010predominant IgM autoantibodies in the first year after Kasai portoenterostomy. Anti\u2010CHI3L1, anti\u2010DLL\u20104, and anti\u2010SFTPD IgM autoantibody correlations with worse outcomes and the detection of C3d on cholangioctyes and antigen\u2010specific autoreactive T cells suggest that autoimmunity plays a role in the ongoing bile duct injury and progression of disease.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.31504","type":"journal-article","created":{"date-parts":[[2020,8,7]],"date-time":"2020-08-07T11:35:19Z","timestamp":1596800119000},"page":"1855-1867","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":17,"title":["Unique Cholangiocyte\u2010Targeted IgM Autoantibodies Correlate With Poor Outcome in Biliary Atresia"],"prefix":"10.1097","volume":"73","author":[{"given":"Yuhuan","family":"Luo","sequence":"first","affiliation":[{"name":"University of Colorado School of Medicine,Children\u2019s Hospital 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