Immunoglobulin G4-Associated Cholangitis

Clinical Profile and Response to Therapy

Amaar Ghazale, Suresh T. Chari, Lizhi Zhang, Thomas C. Smyrk, Naoki Takahashi, Michael J. Levy, Mark D. Topazian, Jonathan E. Clain, Randall K. Pearson, Bret T. Petersen, Santhi Swaroop Vege, Keith Lindor, Michael B. Farnell

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)706-715
Number of pages10
JournalGastroenterology
Volume134
Issue number3
DOIs
StatePublished - Mar 2008
Externally publishedYes

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Cholangitis
Immunoglobulins
Steroids
Recurrence
Pathologic Constriction
Immunoglobulin G
Bile Ducts
Therapeutics
Pancreatic Diseases
Obstructive Jaundice
Serum
Pharmaceutical Preparations
Pancreatitis
Stents
Biopsy
Liver

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Ghazale, A., Chari, S. T., Zhang, L., Smyrk, T. C., Takahashi, N., Levy, M. J., ... Farnell, M. B. (2008). Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy. Gastroenterology, 134(3), 706-715. https://doi.org/10.1053/j.gastro.2007.12.009

Immunoglobulin G4-Associated Cholangitis : Clinical Profile and Response to Therapy. / Ghazale, Amaar; Chari, Suresh T.; Zhang, Lizhi; Smyrk, Thomas C.; Takahashi, Naoki; Levy, Michael J.; Topazian, Mark D.; Clain, Jonathan E.; Pearson, Randall K.; Petersen, Bret T.; Vege, Santhi Swaroop; Lindor, Keith; Farnell, Michael B.

In: Gastroenterology, Vol. 134, No. 3, 03.2008, p. 706-715.

Research output: Contribution to journalArticle

Ghazale, A, Chari, ST, Zhang, L, Smyrk, TC, Takahashi, N, Levy, MJ, Topazian, MD, Clain, JE, Pearson, RK, Petersen, BT, Vege, SS, Lindor, K & Farnell, MB 2008, 'Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy', Gastroenterology, vol. 134, no. 3, pp. 706-715. https://doi.org/10.1053/j.gastro.2007.12.009
Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ et al. Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy. Gastroenterology. 2008 Mar;134(3):706-715. https://doi.org/10.1053/j.gastro.2007.12.009
Ghazale, Amaar ; Chari, Suresh T. ; Zhang, Lizhi ; Smyrk, Thomas C. ; Takahashi, Naoki ; Levy, Michael J. ; Topazian, Mark D. ; Clain, Jonathan E. ; Pearson, Randall K. ; Petersen, Bret T. ; Vege, Santhi Swaroop ; Lindor, Keith ; Farnell, Michael B. / Immunoglobulin G4-Associated Cholangitis : Clinical Profile and Response to Therapy. In: Gastroenterology. 2008 ; Vol. 134, No. 3. pp. 706-715.
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AU - Takahashi, Naoki

AU - Levy, Michael J.

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