New Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis.

Abstract

Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the multisystem IgG4-related disease. IgG4-SC presents with biliary strictures and/or masses that can bear a striking similarity to other malignant and inflammatory diseases. Diagnosis is based on a combination of clinical, biochemical, radiological, and histologic findings with careful exclusion of malignant disease. Corticosteroids are the mainstay of treatment with good clinical, biochemical, and radiological responses. This review provides a comprehensive overview of the current knowledge of the prevalence, clinical features, radiology and histology findings, diagnosis, treatment, natural history, and pathophysiology of IgG4-SC.

• Authors: Wouter L Smit, Emma L Culver, Roger W Chapman
• Journal: Clinics in liver disease (Clin Liver Dis) Vol. 20 Issue 1 Pg. 47-65 (Feb 2016) ISSN: 1557-8224 [Electronic] United States
• PMID: 26593290 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2016 Elsevier Inc. All rights reserved.

Chemical References

• Chemokines
• Immunoglobulin G

Topics

• Autoimmune Diseases (diagnosis, drug therapy, immunology)
• Chemokines (metabolism)
• Cholangitis, Sclerosing (diagnosis, drug therapy, immunology)
• Humans
• Immunoglobulin G (immunology)
• Prognosis
• T-Lymphocytes, Helper-Inducer (immunology)
• T-Lymphocytes, Regulatory (immunology)