Diagnosis and management of pulmonary hypertension in systemic sclerosis.
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.
Sweiss
Nadera J
NJ
Section of Rheumatology, University of Chicago, 5841 South Maryland Avenue (MC6080), Chicago, IL 60637, USA.
Hushaw
Linda
L
Thenappan
Thenappan
T
Sawaqed
Ray
R
Machado
Roberto F
RF
Patel
Amit R
AR
Gomberg-Maitland
Mardi
M
Husain
Aliya N
AN
Archer
Stephen L
SL
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
D000959:Antihypertensive Agents