TY - JOUR
T1 - Diagnosis and management of pulmonary hypertension in systemic sclerosis
AU - Sweiss, Nadera J.
AU - Hushaw, Linda
AU - Thenappan, Thenappan
AU - Sawaqed, Ray
AU - MacHado, Roberto F.
AU - Patel, Amit R.
AU - Gomberg-Maitland, Mardi
AU - Husain, Aliya N.
AU - Archer, Stephen L.
PY - 2010/2
Y1 - 2010/2
N2 - 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.
AB - 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.
KW - Circulating autoantibodies
KW - Connective tissue disease
KW - Endothelin receptor antagonists
KW - Flolan (epoprostenol)
KW - Phosphodiesterase 5 inhibitors
KW - Pulmonary arterial hypertension
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U2 - 10.1007/s11926-009-0078-1
DO - 10.1007/s11926-009-0078-1
M3 - Review article
C2 - 20425528
AN - SCOPUS:77249095292
SN - 1523-3774
VL - 12
SP - 8
EP - 18
JO - Current rheumatology reports
JF - Current rheumatology reports
IS - 1
ER -