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

作者：Kudo Tanaka Eriko^*; Nakatsuka Shinichi; Hirano Toru; Kawai Mari; Katada Yoshinori; Matsushita Masato; Ohshima Shiro; Ishii Masaru; Miyatake Kunio; Tanaka Toshio; Saeki Yukihiko

来源：Modern Rheumatology, 2009, 19(6): 691-695.

DOI：10.1007/s10165-009-0214-9

• Mikulicz's disease
• Immunoglobulin G4 (IgG4)
• CD40 ligation
• Th2
• Sjogren's syndrome

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-gamma) compared with patients with Sjogren'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.

• 出版日期2009-12