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Possible involvement of T helper type 2 responses to Toll-like receptor ligands in IgG4-related sclerosing disease
  1. Reiko Akitake,
  2. Tomohiro Watanabe,
  3. Chikage Zaima,
  4. Norimitsu Uza,
  5. Hiroshi Ida,
  6. Shinsuke Tada,
  7. Naoshi Nishida,
  8. Tsutomu Chiba
  1. Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
  1. Correspondence to Dr Tomohiro Watanabe, Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan; tmhrwtnb{at}kuhp.kyoto-u.ac.jp

Abstract

We report a case of immunoglobulin G4 (IgG4)-related sclerosing disease involving the pancreas, liver and salivary glands. Massive infiltration of IgG4-expressing plasma cells was seen in the liver and submandibular lymph nodes. Interestingly, accumulation of IgG4-expressing plasma cells was also seen in the colon and terminal ileum. Peripheral blood mononuclear cells (PBMCs) isolated from this patient exhibited enhanced production of IgG4 and interleukin-10 upon stimulation with Toll-like receptor (TLR) ligands as compared with those from a healthy control. In contrast, production of tumour necrosis factor α and interferon γ by PBMCs from this patient was markedly reduced. Since colonic mucosa is always exposed to TLR ligands derived from commensal organisms, the results of immunological studies suggest that enhanced T helper type 2 responses to intestinal microflora may underlie the immunopathogenesis in this patient with IgG4-related sclerosing disease.

  • IgG4
  • immunology
  • pancreatic pathology
  • pancreatitis
  • Th2
  • TLR

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Footnotes

  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the Kyoto University review board.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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