Diagnosis and Management of Pulmonary Hypertension in Systemic Sclerosis
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Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH; mean pulmonary artery pressure ≥ 25 mm Hg) caused by pulmonary arterial hypertension (PAH), left ventricular disease, or pulmonary fibrosis. PAH is a pulmonary vascular disease, the diagnosis of which requires pulmonary capillary wedge pressure less than 15 mm Hg, pulmonary vascular resistance greater than 3 Wood Units, and exclusion of thromboembolism and parenchymal lung disease. Molecular mechanisms underlying PAH-SSc include activation of inflammatory and fibrogenic pathways in the vasculature and right ventricle. Circulating autoantibodies trigger endothelial damage and fibroblast activation. PAH most commonly occurs as a late complication in patients with limited cutaneous disease and anticentromere antibodies. Although echocardiography is a useful screening tool, heart catheterization is required to diagnose PAH before initiating therapy. Prognosis and therapeutic response are worse in PAH-SSc than in other PAH categories (median survival, 1–3 y). Approved therapies include prostacyclins, endothelin antagonists, and phosphodiesterase type 5 inhibitors. Research is needed to define disease mechanisms and develop effective therapies.
KeywordsCirculating autoantibodies Pulmonary arterial hypertension Connective tissue disease Phosphodiesterase 5 inhibitors Endothelin receptor antagonists Flolan (epoprostenol)
The authors would like to thank Dr Stuart Rich for his thoughtful review and advice.
Dr. Archer reports no potential conflicts of interest relevant to this article.
[AU: ONLY ARTICLES FROM 2007 TO THE PRESENT CAN BE ANNOTATED -- ANNOTATIONS FOR ARTICLES BEFORE 2007 HAVE THEREFORE BEEN REMOVED]
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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