title: A case of Mikulicz's disease with Th2-biased cytokine profile: possible feature discriminable from Sjögren's syndrome.
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-gamma) compared with patients with Sjögren'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.
authors.affiliation: Department of Rheumatology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan. 04rher@ommc-hp.jp
authors.forename: Eriko
authors.initials: E
authors.lastname: Kudo-Tanaka
authors.forename: Shinichi
authors.initials: S
authors.lastname: Nakatsuka
authors.forename: Toru
authors.initials: T
authors.lastname: Hirano
authors.forename: Mari
authors.initials: M
authors.lastname: Kawai
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Katada
authors.forename: Masato
authors.initials: M
authors.lastname: Matsushita
authors.forename: Shiro
authors.initials: S
authors.lastname: Ohshima
authors.forename: Masaru
authors.initials: M
authors.lastname: Ishii
authors.forename: Kunio
authors.initials: K
authors.lastname: Miyatake
authors.forename: Toshio
authors.initials: T
authors.lastname: Tanaka
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Saeki
mesh_terms: D019013:CD40 Antigens / Q000276:immunology; D023201:CD40 Ligand / Q000276:immunology; D016207:Cytokines / Q000276:immunology*; D003937:Diagnosis, Differential; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood / Q000276:immunology; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D007963:Leukocytes, Mononuclear / Q000276:immunology; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000175:diagnosis* / Q000276:immunology; D012469:Salivary Glands / Q000276:immunology; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000276:immunology; D018418:Th2 Cells / Q000276:immunology*
chemical_list: D019013:CD40 Antigens; D016207:Cytokines; D007074:Immunoglobulin G; D023201:CD40 Ligand; D007073:Immunoglobulin E