PT - JOURNAL ARTICLE AU - Heather Gladue AU - Virginia Steen AU - Yannick Allanore AU - Rajeev Saggar AU - Rajan Saggar AU - Paul Maranian AU - Veronica J. Berrocal AU - Jerome Avouac AU - Christophe Meune AU - Mona Trivedi AU - Dinesh Khanna TI - Combination of Echocardiographic and Pulmonary Function Test Measures Improves Sensitivity for Diagnosis of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: Analysis of 2 Cohorts AID - 10.3899/jrheum.130400 DP - 2013 Oct 01 TA - The Journal of Rheumatology PG - 1706--1711 VI - 40 IP - 10 4099 - http://www.jrheum.org/content/40/10/1706.short 4100 - http://www.jrheum.org/content/40/10/1706.full SO - J Rheumatol2013 Oct 01; 40 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.