title: Connective tissue disease-associated pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) is characterized by vascular remodeling of pulmonary arterioles that leads to increased pulmonary vascular resistance, right heart failure, and death. It is associated with connective tissue diseases, including systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. PAH is characterized by dyspnea on exertion and fatigue. Syncopal events suggest severe disease. Patients may present with signs of right heart failure. One- and 3-year survival rates are approximately 81% and 52%, respectively. Given the high prevalence and mortality, algorithms for screening are currently under investigation and will hopefully lead to earlier diagnosis and improved survival.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA.
authors.forename: Yon K
authors.initials: YK
authors.lastname: Sung
authors.affiliation: Division of Rheumatology and Immunology, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA; Division of Rheumatology, VA Palo Alto Health Care System, 3801 Miranda Avenue, Palo Alto, CA 94304, USA. Electronic address: shauwei@stanford.edu.
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D008403:Mass Screening
keywords: Connective tissue disease; Mixed connective tissue disease; Pulmonary arterial hypertension; Pulmonary hypertension; Systemic lupus erythematosus; Systemic sclerosis