The Geoepidemiology and Clinical Aspects of IgG4-Related Disease

Kazushige Uchida, Toshihiro Tanaka, M. Eric Gershwin, Kazuichi Okazaki

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)187-199
Number of pages13
JournalSeminars in Liver Disease
Issue number3
StatePublished - Aug 1 2016


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

ASJC Scopus subject areas

  • Hepatology


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