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Cited by 890IgG4-Related and Inflammatory DiseasesLiver Diseases and ImmunityGallbladder and Bile Duct Disorders

Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy

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Ghazale, Amaar, Chari, Suresh T., Zhang, Lizhi, Smyrk, Thomas C., Takahashi, Naoki, Levy, Michael J., Topazian, Mark D., Clain, Jonathan E. +5 more·2008·Gastroenterology
DOI 10.1053/j.gastro.2007.12.009
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Abstract

Background & aims Immunoglobulin (Ig)G4-associated cholangitis (IAC) is the biliary manifestation of a steroid-responsive multisystem fibroinflammatory disorder in which affected organs have a characteristic lymphoplasmacytic infiltrate rich in IgG4-positive cells. We describe clinical features, treatment response, and predictors of relapse in IAC and compare relapse rates in IAC with intrapancreatic vs proximal bile duct strictures.Methods We reviewed clinical, serologic, and imaging characteristics and treatment response in 53 IAC patients.Results IAC patients generally were older (mean age, 62 y) men (85%), presenting with obstructive jaundice (77%) associated with autoimmune pancreatitis (92%), increased serum IgG4 levels (74%), and abundant IgG4-positive cells in bile duct biopsy specimens (88%). At presentation, biliary strictures were confined to the intrapancreatic bile duct in 51%; the proximal extrahepatic/intrahepatic ducts were involved in 49%. Initial presentation was treated with steroids (n = 30; median follow-up period, 29.5 months), surgical resection (n = 18; median follow-up period, 58 months), or was conservative (n = 5; median follow-up period, 35 months). Relapses occurred in 53% after steroid withdrawal; 44% relapsed after surgery and were treated with steroids. The presence of proximal extrahepatic/intrahepatic strictures was predictive of relapse. Steroid therapy normalized liver enzyme levels in 61%; biliary stents could be removed in 17 of 18 patients. Fifteen patients treated with steroids for relapse after steroid withdrawal responded; 7 patients on additional immunomodulatory drugs remain in steroid-free remission (median follow-up period, 6 months).Conclusions IAC should be suspected in unexplained biliary strictures associated with increased serum IgG4 and unexplained pancreatic disease. Relapses are common after steroid withdrawal, especially with proximal strictures. The role of immunomodulatory drugs for relapses needs further study.

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  • Involvement of perihilar and intrahepatic bile ducts is associated with higher relapse rates in IRC.

    “Involvement of perihilar and intrahepatic bile ducts has been associated with higher relapse rates and may warrant sustained immunosuppressive therapy.39”

    From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [39]

  • Treatment response to corticosteroids is regarded as a major diagnostic criterion for IRC.

    “Treatment response to corticosteroids is regarded as a major diagnostic criterion for IRC.3,39”

    From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [39]

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  1. Takahashi N, Kawashima A, Fletcher JG, et al. Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 2007;242:791-801.

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Authors
Ghazale, A., Chari, S. T., Zhang, L., Smyrk, T. C., Takahashi, N., Levy, M. J., Topazian, M. D., Clain, J. E., Pearson, R. K., Petersen, B. T., Vege, S. S., Lindor, K., & Farnell, M. B.
Affiliations
Mayo Clinic (US)
Corresponding author
Chari, Suresh T.
ORCID
Venue
Gastroenterology
Publisher
Elsevier BV
Published
2008-03
Volume
134
Issue
3
Pages
706-715
ISSN
0016-5085 (ISSN-L)
DOI
10.1053/j.gastro.2007.12.009
OpenAlex
W2019122570
Cited by
890
References
36
Citations by year
2026: 21 · 2025: 23 · 2024: 28 · 2023: 31 · 2022: 27 · 2021: 44 · 2020: 48 · 2019: 42 · 2018: 71 · 2017: 42 · 2016: 46 · 2015: 78 · 2014: 70 · 2013: 85 · 2012: 72
FWCI
36.59
Type
journal article
Language
en
Open access
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • Gastroenterology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
  • Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Gallbladder and Bile Duct Disorders
Keywords
IgG4-associated cholangitis, biliary strictures, autoimmune pancreatitis, IgG4-positive cells, relapse after steroid withdrawal, corticosteroid therapy, immunomodulators, extrahepatic bile duct stricture, obstructive jaundice, serum IgG4, surgical resection, biliary stent, steroid-free remission
MeSH terms
Adolescent, Adult, Aged, Aged, 80 and over, Autoimmune Diseases, Bile Ducts, Biliary Tract Surgical Procedures, Cholangiography, Female, Follow-Up Studies, Humans, Immunoglobulin G, Immunohistochemistry, Immunologic Factors, Male, Middle Aged, Pancreatitis, Recurrence, Steroids, Time Factors, Cholangitis, Sclerosing, Cohort Studies, Up-Regulation, Treatment Outcome, Disease-Free Survival, Jaundice, Obstructive, Kaplan-Meier Estimate

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