title: Measuring outcomes in PAH: the gap between the measures that are used and their validity.
abstract: Pulmonary arterial hypertension (PAH) has poor prognosis in systemic sclerosis (SSc), and at present the "gold standard" for diagnosis and follow-up of PAH in SSc is right heart catheterization (RHC) but it would be very useful to have a noninvasive way to follow these patients. Using the OMERACT criteria for validation of measures of response, the only fully validated measure for PAH in SSc has been the 6-min walking test. Multiple other measures are particularly valid (e.g., echocardiography, brain natriuretic protein [BNP], FV/DLCO) while a few are unlikely ever to be validated for various reasons (e.g., symptoms, MRA). A Delphi exercise among 78 experts (EPOSS) has been done and has developed a consensus document consisting of eight domains (lung vascular/PAP, exercise capacity, cardiac function, dyspnea, discontinuation of treatment, quality of life, lung parenchymal, and global state), which can be used and must be tested.
authors.affiliation: Geffen School of Medicine at the University of California in Los Angeles, 1000 Veteran Avenue Room 32-59, Los Angeles, CA 90095, USA. defurst@mednet.ucla.edu
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
mesh_terms: D001158:Arteries / Q000503:physiopathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology* / Q000628:therapy*; D016896:Treatment Outcome