title: Diagnosis and management of pulmonary hypertension in systemic sclerosis.
abstract: 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.
authors.affiliation: Section of Rheumatology, University of Chicago, 5841 South Maryland Avenue (MC6080), Chicago, IL 60637, USA.
authors.forename: Nadera J
authors.initials: NJ
authors.lastname: Sweiss
authors.forename: Linda
authors.initials: L
authors.lastname: Hushaw
authors.forename: Thenappan
authors.initials: T
authors.lastname: Thenappan
authors.forename: Ray
authors.initials: R
authors.lastname: Sawaqed
authors.forename: Roberto F
authors.initials: RF
authors.lastname: Machado
authors.forename: Amit R
authors.initials: AR
authors.lastname: Patel
authors.forename: Mardi
authors.initials: M
authors.lastname: Gomberg-Maitland
authors.forename: Aliya N
authors.initials: AN
authors.lastname: Husain
authors.forename: Stephen L
authors.initials: SL
authors.lastname: Archer
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D005080:Exercise Test; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology*; D012595:Scleroderma, Systemic / Q000150:complications*; D014463:Ultrasonography
chemical_list: D000959:Antihypertensive Agents