The role and mechanism of cathepsin G in dermatomyositis.

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by CD4+ T cells and B cells infiltration in perivascular and muscle tissue. Although the infiltration of inflammatory cells plays a key role in muscle damage, the exact mechanism is not clear. Cathepsin G (CTSG) is a member of the serine proteases family and can increase the permeability of vascular endothelial cells and the chemotaxis of inflammatory cells. In this study, we found that the expression of CTSG was increased in peripheral blood mononuclear cells and muscle tissues of DM patients. The activity of CTSG was significantly increased in DM patients and correlated with disease activity. Serum CTSG induced the expression of protease activated receptor 2 (PAR2) and altered the cytoskeleton of human dermal microvascular endothelial cells. Our studies indicate, for the first time, that CTSG may play an important role in muscle inflammatory cells infiltration by increasing the permeability of vascular endothelial cells.