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Validation of the reveal prognostic equation and risk score calculator in incident systemic sclerosis-associated pulmonary arterial hypertension

Arthritis & Rheumatology May 15, 2019

Mullin CJ, et al. - Given the utility of a prognostic equation and risk score calculator derived from the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) in predicting one-year survival in pulmonary arterial hypertension (PAH), researchers investigated their performance in systemic sclerosis (SSc) associated PAH (SSc-PAH). The predictive accuracy of the REVEAL prediction models was evaluated using prospectively gathered data from the Johns Hopkins Pulmonary Hypertension Program (JHPHP) and Pulmonary Hypertension Assessment and Recognition of Outcome in Scleroderma (PHAROS) Registries. Outcomes suggest that in newly diagnosed SSc-PAH, the REVEAL prognostic equation and risk score provide very good discrimination but poor calibration in predicting one-year survival. They recommend practicing caution while interpreting REVEAL prediction scores in newly diagnosed SSc-PAH patients, particularly those with higher predicted risk resulting from a low six-minute walk test or a high brain natriuretic peptide.
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