RT Journal Article
SR Electronic
T1 Combination of Echocardiographic and Pulmonary Function Test Measures Improves Sensitivity for Diagnosis of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: Analysis of 2 Cohorts
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1706
OP 1711
DO 10.3899/jrheum.130400
VO 40
IS 10
A1 Heather Gladue
A1 Virginia Steen
A1 Yannick Allanore
A1 Rajeev Saggar
A1 Rajan Saggar
A1 Paul Maranian
A1 Veronica J. Berrocal
A1 Jerome Avouac
A1 Christophe Meune
A1 Mona Trivedi
A1 Dinesh Khanna
YR 2013
UL http://www.jrheum.org/content/40/10/1706.abstract
AB Objective. To evaluate routinely collected non-invasive tests from 2 systemic sclerosis (SSc) cohorts to determine their predictive value alone and in combination versus right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH). Methods. We evaluated 2 cohorts of patients who were at risk or with incident PAH: (1) The Pulmonary Hypertension Assessment and Recognition Outcomes in Scleroderma (PHAROS) cohort and (2) an inception SSc cohort at Cochin Hospital, Paris, France. Estimated right ventricular systolic pressure (eRVSP) as determined by transthoracic echocardiogram (TTE) and pulmonary function test (PFT) measures was evaluated, and the predictive values determined. We then evaluated patients with PAH missed on TTE cutoffs that were subsequently identified by a PFT measure. Results. In the PHAROS cohort (n = 206), 59 (29%) had RHC-defined PAH. An eRVSP threshold of 35–50 mm Hg failed to diagnose PAH in 7% to 31% of patients, 50% to 70% of which (n = 2–13) were captured by PFT measures. In the Cochin cohort (n = 141), 10 (7%) patients had RHC confirmed PAH. An eRVSP threshold of 35–50 mm Hg missed 0% to 70% (n = 0–7) of patients, of which 0% to 68% (n = 0–6) were met by PFT measures. The combination of TTE and PFT improved the negative predictive value for diagnosing PAH. Conclusion. In 2 large SSc cohorts, screening with TTE and PFT captured a majority of patients with PAH. TTE and PFT complement each other for the diagnosis of PAH.