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

Abstract

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.

Keywords::

• Autoimmune pancreatitis
• retroperitoneal fibrosis
• salivary gland
• IL-13

Acknowledgments

This study was supported by Japan Society for the Promotion of Science Grant Number 25462675 (Grant in Aids for Scientific Research C). We wish to express our sincere thanks to Mis. Yuko Ohta, Uyo Gakuen college, for her technical advice.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.