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Part VI. Autoantibodies and the Lung |
Measuring Outcomes in PAHThe Gap between the Measures That Are Used and Their Validity
DANIEL E. FURSTa
a Geffen School of Medicine at the University of California in Los Angeles, Los Angeles, California, USA
Key Words: pulmonary arterial hypertension • systemic sclerosis • outcome measurements
Address for correspondence: Daniel E. Furst, M.D., Professor of Rheumatology, Geffen School of Medicine at the University of California in Los Angeles, 1000 Veteran Avenue Room 32-59, Los Angeles, CA 90095. Voice: 310-794-9504; fax: 310-206-8476. defurst{at}mednet.ucla.edu
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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