Roles of TGF-beta and periostin in fibrosclerosis in patients with IgG4-related diseases

Acta Otolaryngol. 2013 Dec;133(12):1322-7. doi: 10.3109/00016489.2013.831187.


Conclusion: These results suggest that transforming growth factor (TGF)-beta and periostin could be useful as novel biomarkers and therapeutic targets in IgG4-related disease.

Objectives: IgG4-related disease is an uncommon fibrosclerosing and inflammatory mass-forming disease that can be systemic or can affect single organs. To clarify the roles of TGF-beta, periostin, and interleukin (IL)-13 in the pathogenesis of IgG4-related disease, we studied a total of 36 serum and 11 tissue samples from patients with IgG4-related disease.

Methods: This was a retrospective clinical study. The patient group consisted of six females and seven males (average age 60 years, range 38-74 years). Serum IgG4 levels, the tissue density of IgG4-positive plasmacytes, and the expression of TGF-beta and periostin in the affected tissues were examined immunohistochemically.

Results: Serum IgG4 levels were elevated in all patients (mean 776.6, range 185-2820 mg/dl), and IgG4-positive plasmacytes were observed in the affected salivary glands. Seven patients with prominent infiltration of the involved glands with IgG4-positive plasmacytes had fatal systemic complications, including pancreatitis, after swelling of the salivary glands. Overexpression of TGF-beta and periostin was observed in affected tissues obtained from these patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Anti-Idiotypic / blood
  • Antibodies, Anti-Idiotypic / immunology*
  • Autoimmune Diseases / blood*
  • Autoimmune Diseases / immunology
  • Cell Adhesion Molecules / biosynthesis*
  • Cell Adhesion Molecules / blood
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G / immunology*
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Pancreatitis / blood*
  • Pancreatitis / immunology
  • Retroperitoneal Fibrosis / blood
  • Retroperitoneal Fibrosis / congenital*
  • Retroperitoneal Fibrosis / immunology
  • Retrospective Studies
  • Transforming Growth Factor beta / biosynthesis*
  • Transforming Growth Factor beta / blood


  • Antibodies, Anti-Idiotypic
  • Cell Adhesion Molecules
  • Immunoglobulin G
  • POSTN protein, human
  • Transforming Growth Factor beta

Supplementary concepts

  • Multifocal fibrosclerosis