Abhishek Gadre1, Christian Ghattas2, Xiaozhen Han3, Xiaofeng Wang3, Omar Minai4, Kristin B Highland5. 1. Internal Medicine, Cleveland Clinic Foundation, Cleveland, OH, USA. 2. Department of Pulmonary and Critical Care, St. Elizabeth Medical Center, Boston, MA, USA. 3. Respiratory Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, A90, Cleveland, OH, 44195, USA. 4. Pulmonary and Critical Care Associates, Petersburg, VA, USA. 5. Respiratory Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, A90, Cleveland, OH, 44195, USA. highlak@ccf.org.
Abstract
BACKGROUND: Pulmonary hypertension (PH) is a common complication of scleroderma (SSc) and is a leading cause of morbidity and mortality. OBJECTIVES: To explore the utility of the 6MWT in the prediction of SSc-PH and to assess its prognostic implications. METHODS: A retrospective review of SSc patients from 2003 to 2013, with 6MWT and echocardiogram (n = 286), was conducted. Presence of PH was defined by right heart catheterization. Patients were randomized into development and validation cohorts. Using regression techniques, we developed a scoring system to predict the presence of SSc-PH and tested it in our validation cohort. Trends of mortality and disease severity were studied for incremental scores. RESULTS: The DIBOSA scoring system includes DIstance walked in 6 min, BOrg dyspnea index, and SAturation of oxygen at 6 min. The DIBOSA score in the development cohort ranged from 0 to 3, resulting in an area of 0.858 (P < 0.0001) under the ROC curve. A score of 0 had a NPV of 100% and a score of 3 had a PPV of 86.58%. The validation cohort had an area under the ROC curve of 0.842. The DIBOSA score correlated with both pulmonary artery pressures and mortality. The 3-year survival rates for DIBOSA scores of 0, 1, 2, and 3 were 100, 100, 87.67, and 66.67%, respectively (HR = 3.92, P < 0.0001). CONCLUSIONS: DIBOSA score is a sensitive tool for the prediction of SSc-PH. The DIBOSA score is a direct predictor of mortality in SSc-PH and strongly correlates with pulmonary pressures. 6MWT can be used to predict clinical outcomes in SSc-PH.
BACKGROUND: Pulmonary hypertension (PH) is a common complication of scleroderma (SSc) and is a leading cause of morbidity and mortality. OBJECTIVES: To explore the utility of the 6MWT in the prediction of SSc-PH and to assess its prognostic implications. METHODS: A retrospective review of SSc patients from 2003 to 2013, with 6MWT and echocardiogram (n = 286), was conducted. Presence of PH was defined by right heart catheterization. Patients were randomized into development and validation cohorts. Using regression techniques, we developed a scoring system to predict the presence of SSc-PH and tested it in our validation cohort. Trends of mortality and disease severity were studied for incremental scores. RESULTS: The DIBOSA scoring system includes DIstance walked in 6 min, BOrg dyspnea index, and SAturation of oxygen at 6 min. The DIBOSA score in the development cohort ranged from 0 to 3, resulting in an area of 0.858 (P < 0.0001) under the ROC curve. A score of 0 had a NPV of 100% and a score of 3 had a PPV of 86.58%. The validation cohort had an area under the ROC curve of 0.842. The DIBOSA score correlated with both pulmonary artery pressures and mortality. The 3-year survival rates for DIBOSA scores of 0, 1, 2, and 3 were 100, 100, 87.67, and 66.67%, respectively (HR = 3.92, P < 0.0001). CONCLUSIONS: DIBOSA score is a sensitive tool for the prediction of SSc-PH. The DIBOSA score is a direct predictor of mortality in SSc-PH and strongly correlates with pulmonary pressures. 6MWT can be used to predict clinical outcomes in SSc-PH.
Entities:
Keywords:
Outcome assessment (health care); Prognosis; Pulmonary hypertension; Systemic scleroderma; Walk test
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