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Semin Liver Dis 2016; 36(03): 187-199
DOI: 10.1055/s-0036-1584323
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

The Geoepidemiology and Clinical Aspects of IgG4-Related Disease

Kazushige Uchida
1   Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka, Japan
,
Toshihiro Tanaka
1   Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka, Japan
2   Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, California
,
M. Eric Gershwin
2   Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, California
,
Kazuichi Okazaki
1   Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka, Japan
› Author Affiliations
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Publication History

Publication Date:
28 July 2016 (online)

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Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a recently described systemic inflammatory disease characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis. However, although IgG4-RD has become increasingly recognized, the number of patients with IgG4-RD remains unclear. Data from several studies indicate that patients who have a T-helper type 2 (Th2-) dominant immune response, which leads to the hyperproduction of Th2 cytokines, then progress to IgG4-RD. Glucocorticoids are the most common treatment for IgG4-RD and generally, patients have a good response—a characteristic of IgG4-RD. However, relapses during the tapering of glucocorticoid therapy are common. Second-line therapy after glucocorticoids includes immunosuppressant agents. Although the long-term outcome still remains unclear, there is increased interest in the relationships between IgG-RD and malignancies. In this review, the authors provide a detailed overview of the geoepidemiology, pathogenesis, diagnostic features, treatment, and prognosis of IgG4-RD.

Keywords

T-helper type 2 - cytokines - geoepidemiology - immunoglobulin G4 - immunoglobulin G4-related disease
 

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