Abstract
Pulmonary arterial hypertension (PAH) is a severe disease with high morbidity and mortality. Current therapies are mainly focused on vasodilative agents to improve prognosis. However, recent literature has shown the important interaction between immune cells and stromal vascular cells in the pathogenic modifications of the pulmonary vasculature. The immunological pathogenesis of PAH is known as a complex interplay between immune cells and vascular stromal cells, via direct contacts and/or their production of extra-cellular/diffusible factors such as cytokines, chemokines, and growth factors. These include, the B-cell—mast-cell axis, endothelium mediated fibroblast activation and subsequent M2 macrophage polarization, anti-endothelial cell antibodies and the versatile role of IL-6 on vascular cells. This review aims to outline the major pathophysiological changes in vascular cells caused by immunological mechanisms, leading to vascular remodeling, increased pulmonary vascular resistance and eventually PAH. Considering the underlying immunological mechanisms, these mechanisms may be key to halt progression of disease.
Highlights
Pulmonary arterial hypertension (PAH) is a progressive cardiovascular disease with high mortality and serious impact on the quality of life of affected patients
This study showed an increase in CD68+ cells in the adventitial vascular layer in human Idiopathic PAH (IPAH), hypoxia induced Pulmonary Hypertension (PH) in calves and MCT rats
This study showed that pulmonary arterial smooth muscle cells (PASMCs) that were harvested from the same vessels, do not show a different phenotype
Summary
Pulmonary arterial hypertension (PAH) is a progressive cardiovascular disease with high mortality and serious impact on the quality of life of affected patients. PAH, known as pre-capillary PH, is defined by a Immunology in Pulmonary Arterial Hypertension mPAP ≥ 20 mmHg and a PAWP ≤ 15 mmHg. The PVR is critical to distinguish post-capillary PH from combined preand post-capillary PH. When studying the etiologies of PH, numerous different underlying diseases may contribute to the development of the typical vasculopathy or vascular remodeling that subsequently leads to increased pressure in the pulmonary circulation. ANCA-associated vasculitis, a systemic disease that predominantly affects small and medium-sized vessels, is almost never complicated by PAH These observations highlight the complexity of PAH and its association with very specific pathological mechanisms leading to vascular remodeling seen in PAH. Possibilities for future research and clinical implications will extensively be discussed
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