Brief communication
Rituximab Therapy for Refractory Biliary Strictures in Immunoglobulin G4–Associated Cholangitis

https://doi.org/10.1016/j.cgh.2007.12.020Get rights and content

Background & Aims: Biliary strictures occur in a third of patients with autoimmune pancreatitis and have been termed immunoglobulin G subclass 4 (IgG4) associated cholangitis (IAC). IAC often responds to steroid therapy. Methods: A patient with autoimmune pancreatitis and (IAC) refractory to steroids and 6-mercaptopurine was treated with rituximab, a monoclonal antibody directed against the CD20 antigen on B lymphocytes. Results: The patient’s biliary strictures improved after rituximab therapy, permitting removal of his biliary stents. Systemic manifestations of IgG4-associated disease also improved. Conclusions: Rituximab may be a treatment option for patients with refractory or recurrent autoimmune pancreatitis or IAC.

Section snippets

Case Report

A 64-year-old man developed pancreatitis in the fall of 2004. Serum liver tests were normal except for an alkaline phosphatase level of 130 U/L (normal, 80–120 U/L). History, laboratory evaluation, abdominal ultrasound, and computerized tomography scan did not reveal an etiology. Magnetic resonance imaging showed an irregular main pancreatic duct with a stenosis in the neck. His serum IgG4 level was 568 mg/dL (normal, 8–140 mg/dL). Endoscopic ultrasound showed a hypoechoic, enlarged pancreas.

Discussion

AIP is an inflammatory disorder characterized by pancreatic enlargement, multifocal pancreatic duct strictures, increased serum IgG4 levels, and a pancreatic IgG4-positive lymphoplasmacytic infiltrate with narrowing of small ducts and obliterative phlebitis. Bile duct strictures are reported in a third of patients with AIP and have been termed IAC.3 IAC also may occur in patients without pancreatitis. Stenoses may occur in the intrapancreatic bile duct, biliary confluence, and/or peripheral

References (19)

There are more references available in the full text version of this article.

Cited by (157)

  • Miscellaneous Interstitial Lung Diseases

    2021, Encyclopedia of Respiratory Medicine, Second Edition
  • Sclerosing cholangitis related to IgG4: Not always a curable entity

    2019, Annals of Hepatology
    Citation Excerpt :

    Liver disease usually involves bile duct structures and, for this reason, IgG4-RD is considered an important cause of secondary sclerosing cholangitis (SSC). IgG4-SC occurs in a third of patients with type 1 autoimmune pancreatitis (AIP),3 but other organ involvement can be possible. IgG4-RD is considered an entity with a relatively good prognosis due to its high rate of response to steroid therapy.4-6

View all citing articles on Scopus
1

Dr Witzig serves on the Genentech and BiogenIDEC advisory boards and receives research support from these companies for clinical trials. This information was disclosed to participants.

View full text