Infection & Immunity
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of multiple organs, preceded by vascular and immune dysfunction.
The mechanisms responsible for the initiation of SSc are unknown, however, our recent work demonstrated that CXCL4 chemokine released by Plasmacytoid Dendritic Cells (pDC) is implicated in many crucial events that go awry in SSc patients.
SSc is linked to hypoxia and caused by vascular damage and ECM accumulation resulting in excessive reactive oxygen species (ROS) formation in mitochondria system. Our data indicate that hypoxia- induced ROS plays a crucial role in the stimulation of pDCs to produce high levels of CXCL4.