Vol.19 No.6

Case Report

A case of Mikulicz’s disease with Th2-biased cytokine profile: possible feature discriminable from Sjögren’s syndrome

Authors

Eriko Kudo-Tanaka1 , Shinichi Nakatsuka2 , Toru Hirano3 , Mari Kawai3 , Yoshinori Katada4 , Masato Matsushita1 , Shiro Ohshima1,5 , Masaru Ishii1 , Kunio Miyatake6 , Toshio Tanaka3 , Yukihiko Saeki1,5

  • Department of Rheumatology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano Osaka, 586-8521, Japan
  • Department of Pathology, NHO Osaka-Minami Medical Center, Kawachinagano, Osaka, Japan
  • Department of Respiratory Medicine Allergy and Rheumatic Diseases, Osaka University Graduate School of Medicine, Osaka, Japan
  • Department of Allergy, NHO Osaka-Minami Medical Center, Kawachinagano, Osaka, Japan
  • Department of Clinical Research, NHO Osaka-Minami Medical Center, Kawachinagano, Osaka, Japan
  • NHO Osaka-Minami Medical Center, Kawachinagano, Osaka, Japan
Received:

26 March 2009

Accepted:

8 July 2009

Published online:

21 August 2009

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Abstract

This article concerns a male patient with Mikulicz’s disease (MD) accompanied with marked elevation of serum immunoglobulin (Ig)G4 and IgE levels. His peripheral blood mononuclear cells (PBMC) showed markedly enhanced in vitro production of interleukin (IL)-4, IL-5, IL-13, but not interferon gamma (IFN-γ) compared with patients with Sj?gren’s syndrome (SS) and healthy donors, suggesting distinct Th2 bias in this MD patient. Besides the prominent infiltration of IgG4-producing plasma cells, the enhanced expression of both CD40 and CD40 ligand (CD40L) were observed in the swollen salivary gland of the MD patient, suggesting enhanced signaling pathways for the induction of IgG4 and IgE switching. Possible differences between MD and SS in light of their underlying pathogenesis are discussed.

Key words

Mikulicz’s disease - Immunoglobulin G4 (IgG4) - CD40 ligation - Th2 - Sjögren’s syndrome