PT - JOURNAL ARTICLE AU - Vivien M. Hsu AU - Lorinda Chung AU - Laura K. Hummers AU - Ami Shah AU - Robert Simms AU - Marcy Bolster AU - Faye N. Hant AU - Richard M. Silver AU - Aryeh Fischer AU - Monique E. Hinchcliff AU - John Varga AU - Avram Z. Goldberg AU - Chris T. Derk AU - Elena Schiopu AU - Dinesh Khanna AU - Lee S. Shapiro AU - Robyn T. Domsic AU - Thomas Medsger AU - Maureen D. Mayes AU - Daniel Furst AU - Mary Ellen Csuka AU - Jerry A. Molitor AU - Lesley Ann Saketkoo AU - Christian R. Salazar AU - Virginia D. Steen TI - Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry AID - 10.3899/jrheum.180018 DP - 2019 Feb 01 TA - The Journal of Rheumatology PG - 176--183 VI - 46 IP - 2 4099 - http://www.jrheum.org/content/46/2/176.short 4100 - http://www.jrheum.org/content/46/2/176.full SO - J Rheumatol2019 Feb 01; 46 AB - Objective. We sought to identify predictors of mortality and cardiopulmonary hospitalizations in patients at risk for pulmonary hypertension (PH) and enrolled in PHAROS, a prospective cohort study to investigate the natural history of PH in systemic sclerosis (SSc).Methods. The at-risk population for PH was defined by the following entry criteria: echocardiogram systolic pulmonary arterial pressure > 40 mmHg, or DLCO < 55% predicted or ratio of % forced vital capacity/%DLCO > 1.6, measured by pulmonary function testing. Baseline clinical measures were evaluated as predictors of hospitalization and death between 2005 and 2014. Cox proportional hazards models were censored at date of PH onset or latest study visit and adjusted for age, sex, race, and disease duration.Results. Of the 236 at-risk subjects who were followed for a median of 4 years (range 0.4–8.5 yrs), 35 developed PH after entering PHAROS (reclassified as PH group). In the at-risk group, higher mortality was strongly associated with male sex, low %DLCO, exercise oxygen desaturation, anemia, abnormal dyspnea scores, and baseline pericardial effusion. Risks for cardiopulmonary hospitalization were associated with increased dyspnea and pericardial effusions, although PH patients with DLCO < 50% had the highest risk of cardiopulmonary hospitalizations.Conclusion. Risk factors for poor outcome in patients with SSc who are at risk for PH were similar to others with SSc-PH and SSc-pulmonary arterial hypertension, including male sex, DLCO < 50%, exercise oxygen desaturation, and pericardial effusions. This group should undergo right heart catheterization and receive appropriate intervention if PH is confirmed.