title: Update in systemic sclerosis-associated pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Despite advances in treatment options for PAH, long-term prognosis remains poor for scleroderma-associated PAH (SSc-PAH). Although prompt diagnosis and treatment of PAH may have significant impact on survival rates, early detection of the syndrome continues to be challenging in SSc due to several factors ranging from limitations of the current screening tools and the complexities of the disease. In comparison with other PAH subgroups, SSc-PAH patients respond poorly to conventional forms of PAH therapy. Recent findings indicate that factors such as autoimmune and inflammatory responses, more severe vasculature remodeling, and intrinsic cardiac involvement may account for these differences.
authors.affiliation: Department of medicine, division of pulmonary and critical care medicine, Johns Hopkins University school of medicine, Saint Luke's hospital, 232 S Woods Mill Road, Baltimore, MD, 21224, United States; Saint Luke's Hospital, 232 S Woods Mill Road, Chesterfield, MO 63017, United States. Electronic address: mohamed.gashouta@stlukes-stl.com.
authors.forename: Mohamed A
authors.initials: MA
authors.lastname: Gashouta
authors.affiliation: University Paris-Sud, Faculté de médecine, 94270 Le Kremlin-Bicêtre, France; Assistance Publique-Hôpitaux de Paris, hôpital Bicêtre, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie, 94275 Le Kremlin-Bicêtre, France; Inserm U999, hypertension artérielle pulmonaire : physiopathologie et innovation thérapeutique, centre chirurgical Marie-Lannelongue, 92350 Le Plessis-Robinson, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Department of medicine, division of pulmonary and critical care medicine, Johns Hopkins University school of medicine, Saint Luke's hospital, 232 S Woods Mill Road, Baltimore, MD, 21224, United States.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Hassoun
mesh_terms: D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012595:Scleroderma, Systemic / Q000150:complications*