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Highly Accessed Editorial

IgG4-related disease: why high IgG4 and fibrosis?

Takao Koike

Author affiliations

Graduate School of Medicine, Hokkaido University, Sapporo, N-15 W-7, Kita-ku, Sapporo, Japan 060-8638

Citation and License

Arthritis Research & Therapy 2013, 15:103  doi:10.1186/ar4122


See related research by Tsuboi et al., http://arthritis-research.com/content/14/4/R171

Published: 25 January 2013

Abstract

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.