Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Current Status and Future Direction

Figure 2

Role of inflammation and Dysregulated immune response in the development of PAH in SLE. (A) Viral infection, AECA, and other agents damage the normal pulmonary endothelium. (B) Increase in chemokine/cytokine concentrations as a result of endothelial injury, leading to recruitment of dendritic cells, mast cells, B cells, and T cells. (C) Infiltration of the small, and medium-sized pulmonary arteries by the dendritic cells, mast cells, B cells and T cells, resulting in dysregulated angiogenesis. AECA: antiendothelial cell antibodies; RANTES: regulated upon activation, normal T cell expressed and secreted; CCL5: chemokine Ligand 5; CX3CLI: chemokine Ligand 1 [Fractalkine]; IL-1: interleukin-1; IL-6: interleukin-6; PDGF: platelet derived growth factor.