IgG4-related disease: why high IgG4 and fibrosis?
Graduate School of Medicine, Hokkaido University, Sapporo, N-15 W-7, Kita-ku, Sapporo, Japan 060-8638
Arthritis Research & Therapy 2013, 15:103 doi:10.1186/ar4122
See related research by Tsuboi et al., http://arthritis-research.com/content/14/4/R171Published: 25 January 2013
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.