title: [Pulmonary hypertension associated with systemic sclerosis].
abstract: Pulmonary hypertension is a rare but well known life-threatening complication of scleroderma and particularly in its limited variant, the CREST syndrome (Calcification, Raynaud phenomenon, Esophageal dysmotility, Sclerodactily, Telangiectasia). The aim of this article is to analyze the available literature and to report the experience of a center for pulmonary vascular diseases. Dyspnea is the main symptom and is frequently severe. Echocardiography is an excellent tool to detect pulmonary hypertension. However, right-heart catheterization is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide. Hemodynamic parameters are less severe in patients with connective tissue diseases perhaps because of an earlier diagnosis. A significant lower proportion of patients presents an acute vasodilator response suggesting an early constitution of irreversible pulmonary vascular lesions. Continuous intravenous epoprostenol therapy seems to be less effective as compared with patients with primitive pulmonary hypertension and does not improve survival. In our experience, immunosuppressive therapy does not improve hemodynamic or clinical data. Novel therapies including oral, sub-cutaneous or inhaled stable prostacyclin analogues and endothelin receptor antagonists are currently evaluated in large placebo-controlled trials.
authors.affiliation: Service de Pneumologie et Réanimation Respiratoire, UPRES EA 2705 Maladies vasculaires pulmonaires , Hôpital Antoine-Béclère, 157, rue de la Porte-de-Trivaux, 92140 Clamart, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Sanchez
authors.forename: Hilario
authors.initials: H
authors.lastname: Nunes
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: Gilles
authors.initials: G
authors.lastname: Garcia
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
mesh_terms: D000959:Antihypertensive Agents / Q000494:pharmacology; D006328:Cardiac Catheterization; D004417:Dyspnea / Q000209:etiology; D004452:Echocardiography; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol / Q000494:pharmacology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D017466:Receptors, Endothelin / Q000502:physiology; D012595:Scleroderma, Systemic / Q000150:complications*
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D017466:Receptors, Endothelin; D011464:Epoprostenol