title: Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension.
abstract: This review summarizes an expanding body of knowledge indicating that failure to resolve inflammation and altered immune processes underlie the development of pulmonary arterial hypertension. The chemokines and cytokines implicated in pulmonary arterial hypertension that could form a biomarker platform are discussed. Pre-clinical studies that provide the basis for dysregulated immunity in animal models of the disease are reviewed. In addition, we present therapies that target inflammatory/immune mechanisms that are currently enrolling patients, and discuss others in development. We show how genetic and metabolic abnormalities are inextricably linked to dysregulated immunity and adverse remodeling in the pulmonary arteries.
authors.affiliation: From the Cardiovascular Institute and Department of Pediatrics (M.R.) and Department of Medicine (M.R.N.), Stanford University School of Medicine, CA; INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson and Université Paris-Sud, School of Medicine, Le Kremlin-Bicêtre (C.G., M.H.); and AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital de Bicêtre, France (M.H.). marlener@stanford.edu.
authors.forename: Marlene
authors.initials: M
authors.lastname: Rabinovitch
authors.affiliation: From the Cardiovascular Institute and Department of Pediatrics (M.R.) and Department of Medicine (M.R.N.), Stanford University School of Medicine, CA; INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson and Université Paris-Sud, School of Medicine, Le Kremlin-Bicêtre (C.G., M.H.); and AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital de Bicêtre, France (M.H.).
authors.forename: Christophe
authors.initials: C
authors.lastname: Guignabert
authors.affiliation: From the Cardiovascular Institute and Department of Pediatrics (M.R.) and Department of Medicine (M.R.N.), Stanford University School of Medicine, CA; INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson and Université Paris-Sud, School of Medicine, Le Kremlin-Bicêtre (C.G., M.H.); and AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital de Bicêtre, France (M.H.).
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: From the Cardiovascular Institute and Department of Pediatrics (M.R.) and Department of Medicine (M.R.N.), Stanford University School of Medicine, CA; INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson and Université Paris-Sud, School of Medicine, Le Kremlin-Bicêtre (C.G., M.H.); and AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital de Bicêtre, France (M.H.).
authors.forename: Mark R
authors.initials: MR
authors.lastname: Nicolls
mesh_terms: D000818:Animals; D015415:Biomarkers; D018925:Chemokines / Q000502:physiology; D016207:Cytokines / Q000502:physiology; D003713:Dendritic Cells / Q000276:immunology; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D007109:Immunity; D007249:Inflammation / Q000150:complications*; D008214:Lymphocytes / Q000276:immunology; D008264:Macrophages / Q000502:physiology; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology
chemical_list: D015415:Biomarkers; D018925:Chemokines; D016207:Cytokines
keywords: hypertension, pulmonary; leukotriene B4