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Comparison of the long-term outcomes between proximal and distal IgG4-related sclerosing cholangitis: A multicenter cohort study

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dc.contributor.authorCho, SH-
dc.contributor.authorSong, TJ-
dc.contributor.authorPark, JS-
dc.contributor.authorYoon, JH-
dc.contributor.authorYang, MJ-
dc.contributor.authorYoon, SB-
dc.contributor.authorLee, JM-
dc.contributor.authorLee, YN-
dc.contributor.authorKim, SH-
dc.contributor.authorChoi, EK-
dc.contributor.authorPark, SW-
dc.contributor.authorOh, D-
dc.contributor.authorPark, DH-
dc.contributor.authorLee, SS-
dc.contributor.authorSeo, DW-
dc.contributor.authorLee, SK-
dc.contributor.authorKim, MH-
dc.date.accessioned2023-06-14T02:52:27Z-
dc.date.available2023-06-14T02:52:27Z-
dc.date.issued2023-
dc.identifier.issn0815-9319-
dc.identifier.urihttp://repository.ajou.ac.kr/handle/201003/25929-
dc.description.abstractBackground and Aims: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is considered a biliary manifestation of IgG4-related diseases. However, there has been a controversy on the clinical outcomes according to the location of the involved bile duct. We therefore compared the clinical outcomes and long-term prognosis of IgG4-SC with proximal bile duct involvement (proximal IgG4-SC) and IgG4-SC with distal bile duct involvement (distal IgG4-SC). Methods: We reviewed the data of patients with IgG4-SC that were prospectively collected at 10 tertiary centers between March 2002 and October 2020. Clinical manifestations, outcomes, association with autoimmune pancreatitis (AIP), steroid-responsiveness, and relapse of IgG4-SC were evaluated. Results: A total of 148 patients (proximal IgG4-SC, n = 59; distal IgG4-SC, n = 89) were analyzed. The median age was 65 years (IQR, 56.25–71), and 86% were male. The two groups were similar in terms of jaundice at initial presentation (51% vs 65%; P = 0.082) and presence of elevated serum IgG4 (66% vs 70%; P = 0.649). The two groups showed significant differences in terms of steroid-responsiveness (91% vs 100%; P = 0.008), association with AIP (75% vs 99%; P = 0.001), and occurrence of liver cirrhosis (9% vs 1%; P = 0.034). During a median follow-up of 64 months (IQR, 21.9–84.7), the cumulative relapse-free survival was significantly different between the two groups (67% vs 79% at 5 years; P = 0.035). Conclusions: Relapse of IgG4-SC frequently occurred during follow-up. Proximal IgG4-SC and distal IgG4-SC had different long-term outcomes in terms of steroid-responsiveness, occurrence of liver cirrhosis, and recurrence. It may be advantageous to determine the therapeutic and follow-up strategies according to the location of bile duct involvement.-
dc.language.isoen-
dc.subject.MESHAged-
dc.subject.MESHAutoimmune Diseases-
dc.subject.MESHAutoimmune Pancreatitis-
dc.subject.MESHCholangitis, Sclerosing-
dc.subject.MESHCohort Studies-
dc.subject.MESHDiagnosis, Differential-
dc.subject.MESHFemale-
dc.subject.MESHHumans-
dc.subject.MESHImmunoglobulin G-
dc.subject.MESHLiver Cirrhosis-
dc.subject.MESHMale-
dc.subject.MESHMulticenter Studies as Topic-
dc.subject.MESHSteroids-
dc.titleComparison of the long-term outcomes between proximal and distal IgG4-related sclerosing cholangitis: A multicenter cohort study-
dc.typeArticle-
dc.identifier.pmid36710432-
dc.subject.keywordAutoimmune disease-
dc.subject.keywordCholangitis-
dc.subject.keywordImmunoglobulin G-
dc.subject.keywordImmunoglobulin G4-related disease-
dc.subject.keywordSclerosing-
dc.contributor.affiliatedAuthorYang, MJ-
dc.type.localJournal Papers-
dc.identifier.doi10.1111/jgh.16136-
dc.citation.titleJournal of gastroenterology and hepatology-
dc.citation.volume38-
dc.citation.number4-
dc.citation.date2023-
dc.citation.startPage648-
dc.citation.endPage655-
dc.identifier.bibliographicCitationJournal of gastroenterology and hepatology, 38(4). : 648-655, 2023-
dc.embargo.liftdate9999-12-31-
dc.embargo.terms9999-12-31-
dc.identifier.eissn1440-1746-
dc.relation.journalidJ008159319-
Appears in Collections:
Journal Papers > School of Medicine / Graduate School of Medicine > Gastroenterology
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