The roles of immune system and autoimmunity in pulmonary arterial hypertension: A review

Abstract

Pulmonary arterial hypertension (PAH) is a chronic disease characterized by increased pulmonary artery pressure which if left untreated, can lead to poor quality of life and ultimately death. It is a group of conditions and includes idiopathic PAH, familial/hereditary PAH and associated PAH. The condition has been studied for many years and its association with the immune system and in particular autoimmunity has been investigated. The mechanisms for the pathobiology of PAH are unclear although research has highlighted the role of adaptive and innate immune systems in its development. Diagnostics and therapeutic approaches range from cytokine treatments to the use of immunomodulating drugs, although there is still scope for improvements in the field. This article discusses the mechanisms linked to PAH, its association with other conditions and recent therapeutic interventions.

Keywords: Autoantibodies; Autoimmunity; Immunity; Immunomodulatory therapy; Inflammation; Pulmonary arterial hypertension.