title: Inflammation in pulmonary hypertension: what we know and what we could logically and safely target first.
abstract: Inﬂammation is important for the initiation and the maintenance of vascular remodeling in most of the animal models of pulmonary arterial hypertension (PAH), and therapeutic targeting of inflammation in these models blocks PAH development. In humans, pulmonary vascular lesions of PAH are the source of cytokine and chemokine production, related to inﬂammatory cell recruitment and lymphoid neogenesis. Circulating autoantibodies to endothelial cells and to ﬁbroblasts have been reported in 10-40% of patients with idiopathic PAH, suggesting a possible role for autoimmunity in the pathogenesis of pulmonary vascular lesions. Current specific PAH treatments have immunomodulatory properties, and some studies have demonstrated a correlation between levels of circulating inﬂammatory mediators and patient survival. New immunopathological approaches to PAH should enable the development of innovative treatments for this severe condition.
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, F-94276, France; INSERM UMR-S 999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, LabEx LERMIT, Le Plessis-Robinson, F-92350, France; Centre Chirurgical Marie Lannelongue, Département de recherche Médicale, Le Plessis-Robinson, F-92350, France.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Cohen-Kaminsky
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, F-94276, France; INSERM UMR-S 999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, LabEx LERMIT, Le Plessis-Robinson, F-92350, France; Centre Chirurgical Marie Lannelongue, Département de recherche Médicale, Le Plessis-Robinson, F-92350, France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Hautefort
authors.affiliation: Pulmonary Hypertension Service, Royal Brompton Hospital, SW3 6NP, UK.
authors.forename: Laura
authors.initials: L
authors.lastname: Price
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, F-94276, France; INSERM UMR-S 999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, LabEx LERMIT, Le Plessis-Robinson, F-92350, France; Centre Chirurgical Marie Lannelongue, Département de recherche Médicale, Le Plessis-Robinson, F-92350, France; AP-HP, DHU TORINO, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Bicêtre, Le Kremlin-Bicêtre, F94270, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, F-94276, France; INSERM UMR-S 999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, LabEx LERMIT, Le Plessis-Robinson, F-92350, France; Centre Chirurgical Marie Lannelongue, Département de recherche Médicale, Le Plessis-Robinson, F-92350, France. Electronic address: frederic.perros@gmail.com.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Perros
mesh_terms: D000818:Animals; D015551:Autoimmunity / Q000276:immunology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000276:immunology*; D007155:Immunologic Factors / Q000276:immunology; D007249:Inflammation / Q000276:immunology*
chemical_list: D007155:Immunologic Factors