Human Endogenous Retrovirus K and Pulmonary Arterial Hypertension
A New Take on a Retro Idea
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Pulmonary arterial hypertension (PAH) is a deleterious disease of the lung vasculature characterized by endothelial dysfunction, medial hypertrophy and proliferation, and eventual occlusion of the pulmonary arterioles. Although a number of genetic and environmental triggers have been described as causative, the specific molecular origins of this vascular disease are still unknown.
Multiple lines of evidence now converge to connect immune dysregulation to PAH in humans and animal models. Previous reports suggest that altered inflammatory dynamics, such as hematopoietic and myeloid expansion, B- and T-cell dysfunction, increased cytokine production, circulating autoantibodies, and tertiary lymphoid tissue neogenesis, may initiate or drive PAH.1 Clinically, a well-established causative link exists between autoimmune connective tissue disorders and PAH. Furthermore, infectious agents, such as Schistosoma mansoni and human immunodeficiency virus (HIV), are known to alter the immune cell repertoire and predispose to the development of PAH. However, a mechanistic understanding of the exogenous or endogenous factors that drive immune dysfunction remains largely undefined.
Prior studies have aimed to characterize the role of exogenous viruses in PAH. For example, HIV-associated PAH has been attributed to decreased CD4+ T-cell function, indicating a role for T-cell–mediated immune dysregulation.2 Separately, HIV-encoded proteins such …