Immune cells and autoantibodies in pulmonary arterial hypertension

Analyses of immunity in pulmonary arterial hypertension (PAH) support the notion that maladaptation of the immune response exists. Altered immunity is an increasingly recognized feature of PAH. Indeed, a delicate balance between immunity and tolerance exists and any disturbance may result in chronic inflammation or autoimmunity. This is suggested by infiltration of various immune cells (e.g. macrophages, T and B lymphocytes) in remodeled pulmonary vessels. In addition, several types of autoantibodies directed against antinuclear antigens, endothelial cells (ECs) and fibroblasts have been found in idiopathic and systemic sclerosis-associated PAH. These autoantibodies may play an important role in EC apoptosis and in the expression of cell adhesion molecules. This review article provides an overview of immunity pathways highlighting their potential roles in pulmonary vascular remodeling in PAH and the possibility of future targeted therapy.