title: [Pulmonary arterial hypertension associated with common diseases: connective-tissue diseases, HIV infection and portal hypertension].
abstract: In France, pulmonary arterial hypertension associated with various diseases (connective-tissue diseases, congenital heart diseases, portal hypertension, HIV infection, consumption of anorectic agents) account for approximately 50% of cases of pulmonary arterial hypertension. As it is shown in idiopathic or familial pulmonary arterial hypertension, these forms of pulmonary arterial hypertension are characterized by pulmonary endothelial dysfunction and intense smooth muscle cell proliferation. Their clinical presentation and therapeutic management are similar to those of idiopathic and familial pulmonary arterial hypertension. However, their prognosis differs greatly: it is poor in scleroderma, and much better in congenital heart diseases.
authors.affiliation: Centre de référence national de l'HTAP, service de pneumologie, UPRES EA2705, hôpital Antoine-Béclère, AP-HP, université Paris XI Sud, 92141 Clamart Cedex, France. olivier.sitbon@abc.aphp.fr
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.forename: Jérôme
authors.initials: J
authors.lastname: Le Pavec
authors.forename: Bruno
authors.initials: B
authors.lastname: Degano
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D005602:France / Q000453:epidemiology; D015658:HIV Infections / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D015994:Incidence; D015995:Prevalence; D011379:Prognosis