title: [Pulmonary arterial hypertension: a flavor of autoimmunity].
abstract: It is admitted that autoimmunity results from a combination of risks such as genetic background, environmental triggers, and stochastic events. Pulmonary arterial hypertension (PAH) shares with the so-called prototypic autoimmune diseases, genetic risk factors, female predominance and sex hormone influence, association with other chronic inflammatory and autoimmune diseases, defects in regulatory T cells function, and presence of autoantibodies. Case reports have been published indicating the beneficial effect of some immunosuppressive and anti-inflammatory therapies in PAH, supporting the potential role of immune mechanisms in the pathophysiology of the disease. In this review, we discuss the current knowledge on autoimmune mechanisms operating in PAH, especially mounting a local autoimmune response inside the pulmonary tissue, namely pulmonary lymphoid neogenesis. A better understanding of the role of autoimmunity in pulmonary vascular remodelling may help develop targeted immunomodulatory strategies in PAH.
authors.affiliation: Université Paris-Sud, faculté de médecine, hôpital de Bicêtre, Le Kremlin-Bicêtre, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Perros
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Sylvia
authors.initials: S
authors.lastname: Cohen-Kaminsky
mesh_terms: D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D015551:Autoimmunity; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000276:immunology*; D007167:Immunotherapy; D019947:Receptors, Interleukin-6 / Q000276:immunology
chemical_list: D019947:Receptors, Interleukin-6