IgG4-related disease: why high IgG4 and fibrosis?

Arthritis Res Ther. 2013 Jan 25;15(1):103. doi: 10.1186/ar4122.


The hallmarks of IgG4-related disease (IgG4-RD) are lymphoplasmacytic tissue infiltration with a predominance of IgG4-positive plasma cells, accompanied by fibrosis, obliterative phlebitis, dacryoadenitis, and elevated levels of IgG4. In a recent issue of Arthritis Research & Therapy, Tsuboi and colleagues demonstrated that regulatory T (Treg) cell-and T helper 2 (Th2) cell-derived cytokines contribute to the pathogenesis of Mikulicz's disease, an activation pathway that appears to be common for IgG4-RD. Additional organ-specific factors may account for the different organ involvement of IgG4-RD.

Publication types

  • Editorial
  • Comment

MeSH terms

  • Female
  • Humans
  • Immunoglobulin Class Switching / physiology*
  • Immunoglobulin G / biosynthesis*
  • Immunoglobulin G / blood*
  • Leukocytes, Mononuclear / metabolism*
  • Male
  • Sjogren's Syndrome / blood*


  • Immunoglobulin G