• Rheum. Dis. Clin. North Am. · May 2015

    Review

    Connective tissue disease-associated pulmonary arterial hypertension.

    • Yon K Sung and Lorinda Chung.
    • 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.
    • Rheum. Dis. Clin. North Am. 2015 May 1; 41 (2): 295-313.

    AbstractPulmonary 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. Published by Elsevier Inc.

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