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.
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Authors | Daniel E Furst |
Journal | Annals of the New York Academy of Sciences
(Ann N Y Acad Sci)
Vol. 1107
Pg. 410-6
(Jun 2007)
ISSN: 0077-8923 [Print] United States |
PMID | 17804569
(Publication Type: Journal Article)
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Topics |
- Arteries
(physiopathology)
- Humans
- Hypertension, Pulmonary
(physiopathology, therapy)
- Treatment Outcome
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