ReviewJournal of gastroenterology2016
IgG4-related sclerosing cholangitis: all we need to know.
Review in Journal of gastroenterology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 1 of them a synthesis that pooled it.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
37 citing papers in PubMed, 1 synthesis or guideline pooled it, 84 citations in OpenAlex.
- Clinical practice guidelines for IgG4-related sclerosing cholangitis.Journal of hepato-biliary-pancreatic sciences · 2019Guideline
- Clinical features, responses to therapy and prognosis of 107 patients with IgG4-related cholangitis: a retrospective study.BMC gastroenterology · 2026Observational
- Jaundice marks immunoglobulin G4-related cholangitis in type 1 autoimmune pancreatitis patients.Therapeutic advances in gastroenterology · 2026Article
- Case Report: A steroid-sparing mechanical management of IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma.Frontiers in immunology · 2026Article
- Diagnostic pitfalls in IgG4-related sclerosing cholangitis presenting as perihilar cholangiocarcinoma: case report with literature review.Frontiers in immunology · 2026Review
- Biliary tract neoplasms from common to rare: diagnosis, assessment, and management.Abdominal radiology (New York) · 2026Review
- Molecular mimicry revisited: novel perspectives on autoimmune disease initiation and progression.Frontiers in immunology · 2026Review
- Secondary sclerosing cholangitis: contemporary etiologies, diagnostic pathways, and treatment strategies for clinicians: a narrative review.Translational gastroenterology and hepatology · 2026Review
- Case Report: A rare case of IgG4-related disease initially manifesting with diarrhea and jaundice.Frontiers in immunology · 2026Article
- IgG4-related cholangitis - distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study.BMC gastroenterology · 2025Article
- IgG4-related disease - focus on digestive system involvement.Frontiers in immunology · 2025Review
- Cholangiocyte Organoids: The New Frontier in Regenerative Medicine for the Study and Treatment of Cholangiopathies.Journal of clinical medicine · 2024Review
- Isolated mass-forming IgG4-related sclerosing cholangitis masquerading as extrahepatic cholangiocarcinoma: A case report.World journal of clinical cases · 2021Article
- Diagnosing Biliary Strictures: Distinguishing IgG4-Related Sclerosing Cholangitis From Cholangiocarcinoma and Primary Sclerosing Cholangitis.Mayo Clinic proceedings. Innovations, quality & outcomes · 2021Review
- Systemic IgG4-Related Disease Masquerading as Cholangiocarcinoma: A Case Report Underscoring the Importance of Medical History.The American journal of case reports · 2021Article
- Hilar cholangiocarcinoma with extensive immunoglobulin G4 reaction.International journal of clinical and experimental pathology · 2021Article
- IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease.Canadian journal of gastroenterology & hepatology · 2021Review
- Usefulness of ultrasonography to assess the response to steroidal therapy for the rare case of type 2b immunoglobulin G4-related sclerosing cholangitis without pancreatitis: A case report.World journal of clinical cases · 2020Article
- A Resected Case of Follicular Cholangitis That Was Positive onInternal medicine (Tokyo, Japan) · 2020Article
- Secondary Sclerosing Cholangitis in Critically Ill Patients: An Underdiagnosed Entity.GE Portuguese journal of gastroenterology · 2020Review
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Our knowledge and experience of IgG4-related sclerosing cholangitis (ISC) have expanded in the last decade. ISC is one of the common organ manifestations of IgG4-related disease (IgG4-RD); approximately 60 % of patients with this systemic condition have ISC in the proximal and/or distal bile ducts. ISC needs to be discriminated from primary sclerosing cholangitis, cholangiocarcinoma, and other rare forms of lymphoplasmacytic cholangiopathy (e.g., follicular cholangitis and sclerosing cholangitis with granulocytic epithelial lesions). Its diagnosis requires a multidisciplinary approach, in which serology, histology, and imaging play crucial roles. Treatments with high-dose corticosteroids typically lead to the rapid and consistent induction of disease remission. Another promising therapeutic approach is B-cell depletion with rituximab. Although disease relapse is relatively common, provided that appropriate treatments are administered, ISC is considered a "benign" disease with a low risk of liver failure and biliary malignancy. Its molecular pathology is characterized by Th2-dominant immune reactions, regulatory T-cell activation, and CCL1-CCR8 interactions. Particular subsets of B cells such as plasmablasts and regulatory B cells also expand. A recent global proteomic study demonstrated that three significantly activated immunological cascades in ISC were all B-cell- or immunoglobulin-related (Fc-gamma receptor-mediated phagocytosis, B-cell receptor signaling pathway, and Fc-epsilon receptor I signaling pathway), suggesting the crucial roles of B cells in the underlying immune reactions. Despite the expansion of our knowledge of the pathophysiology of ISC, the exact role of IgG4 remains unclear. A better understanding of its immunopathology will offer some potential drug targets for this emerging biliary disease.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.