ArticleJournal of gastroenterology2020
The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience.
Article in Journal of gastroenterology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 25 citations in OpenAlex.
- [Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021)].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2022Guideline
- A Dual-Gene Signature of PMAIP1 and GADD45A for Early Detection of Intrahepatic Cholangiocarcinoma in the Context of Primary Sclerosing Cholangitis.International journal of molecular sciences · 2026Article
- Hepatic Decompensation Caused by IgG4-positive Plasma Cell Infiltration Mimicking Sclerosing Cholangitis, Who Achieved a Four-year Survival by Glucocorticoid Treatment.Internal medicine (Tokyo, Japan) · 2026Article
- A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma.Journal of liver cancer · 2026Article
- Autoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.Hepatology international · 2026Review
- Clinical features, responses to therapy and prognosis of 107 patients with IgG4-related cholangitis: a retrospective study.BMC gastroenterology · 2026Observational
- Secondary sclerosing cholangitis: contemporary etiologies, diagnostic pathways, and treatment strategies for clinicians: a narrative review.Translational gastroenterology and hepatology · 2026Review
- IgG4-Related Cholangitis.Seminars in liver disease · 2025Review
- [Clinical characteristics and prognosis analysis of patients with IgG4-related hepatobiliary-pancreatic disease].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2024Article
- Biliary fibrosis is an important but neglected pathological feature in hepatobiliary disorders: from molecular mechanisms to clinical implications.Medical review (2021) · 2024Review
- Progress in the Management of Patients with Cholestatic Liver Disease: Where Are We and Where Are We Going?Journal of clinical and translational hepatology · 2024Review
- [Advances in gut microbiomes and immunology of IgG4-related hepatobiliary and pancreatic diseases].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2022Review
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Authors and funding
12 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe long-term outcomes of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are not well known.
methodsThe outcomes of patients with IgG4-SC at Mayo Clinic (1999-2018) were compared to an age- and gender-matched (1:1 ratio) group of patients with primary sclerosing cholangitis (PSC).
resultsWe identified 89 patients with IgG4-SC; median age at diagnosis was 67 years, 81% were males, and the median follow-up was 5.7 years. Seventy-eight patients received prednisone for induction of remission, and 53 received at least one other immunosuppressive agent for maintenance of remission. Of the IgG4-SC group, 10 died (median time from diagnosis until death was 6.5 years): 2 due to cirrhosis, 3 due to cholangiocarcinoma (CCA), and 5 due to non-hepatobiliary causes. Eleven patients in the PSC group underwent liver transplantation, while none did in the IgG4-SC group. The incidence of a hepatobiliary adverse event (cirrhosis or CCA) was 3.4 times greater in the PSC compared to the IgG4-SC group (events per 1000 person-years: 52.6; 95% CI 38-73; vs. 15.6; 95% CI 7-32). The probability of development of a hepatobiliary adverse event within 10 years was 11% in the IgG4-SC compared to 45% in the PSC group (P = 0.0001). The overall survival tended to be higher in the IgG4-SC compared to the PSC group (10-year: 79% vs. 68%, respectively; P = 0.11).
conclusionsIn a cohort of IgG4-SC patients, 88% of whom were treated with immunosuppressive drugs, the risk of cirrhosis and CCA was significantly lower compared to an age- and gender-matched group with PSC.
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