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PHAROS is a prospective cohort studying the natural history of pulmonary hypertension (PH) in systemic sclerosis (SSc). They have shown that risk factors for poor outcomes in this cohort included male sex, DLCO < 50%, exercise oxygen desaturation, and pericardial effusions.
They identified 236 at-risk for PH patients from this population (ECHO systolic pulmonary arterial pressure > 40 mmHg, or DLCO < 55% predicted or ratio of % forced vital capacity/%DLCO > 1.6, measured by pulmonary function testing).
This cohort was followed for 4 years (range 0.4–8.5 yrs), and 35/236 (15%) developed PH after entry (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.
PH patients with DLCO < 50% had the highest risk of cardiopulmonary hospitalizations. Other risks for cardiopulmonary hospitalization were \increased dyspnea and pericardial effusions.
Identification of this at risk group should lead to closer monitoring, ECHO and possibly right heart catheterization to receive appropriate intervention.