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AAT: alpha-1 antitrypsin CCL11: eotaxin-1 CGRP: calcitonin gene-related peptide COPD: chronic obstructive pulmonary disease CS: cigarette smoke CSE: cigarette smoke extract CVD: cardiovascular disease ECs: endothelial cells FEV 1 : forced expiratory volume in 1 s FMD: flow-mediated dilatation GOLD: Global Initiative for Chronic Obstructive Lung Disease HIF-1α: hypoxia-inducible factor 1α ICAM-1: intracellular adhesion molecule-1 ICS: inhaled corticosteroids IL: interleukin MAC-1: macrophage-1 antigen NK: neurokinin receptors NLRP3: Nod-like receptor pyrin domain-containing protein 3 NO: nitric oxide PAT: peripheral arterial tonometry ROS: reactive oxygen species TEM: transendothelial migration VEGF: vascular endothelial growth factors VOP: venous occlusion plethysmography SS: Conceptualization, Writing—original draft

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Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic manifestations (RVCL-S): An update on basic science and clinical perspectives