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Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary
Vivien M Hsu1,2, Lorinda Chung3,4, Laura K Hummers3,4
1From the Department of Medicine, Division of Rheumatology, Rutgers-Robert Wood Johnson Medical School, New Brunswick, New Jersey; Department of Medicine, Division of Immunology and Rheumatology, Stanford University, Stanford, California; Department of Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland; Department of Medicine, Division of Rheumatology, Boston University, Boston; Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, Massachusetts; Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; Department of Medicine, Division of Rheumatology, and Pulmonary Sciences and Critical Care Medicine, University of Colorado, Denver, Colorado; Department of Medicine, Division of Rheumatology, Northwestern University, Chicago, Illinois; Department of Medicine, Division of Rheumatology, New York University Medical Center, New York, New York; Department of Medicine, Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, Michigan; Department of Medicine, Division of Rheumatology, Albany Medical College, Albany, New York; Department of Medicine, Division of Rheumatology, University of Pittsburgh, Pittsburgh, Pennsylvania; Department of Medicine, Division of Rheumatology, University of Texas, Houston, Texas; Department of Medicine, Division of Rheumatology, University of California at Los Angeles, Los Angeles, California; Department of Medicine, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, Wisconsin; Department of Medicine, Division of Rheumatology, University of Minnesota, Minneapolis, Minnesota; Department of Medicine, Divisions of Pulmonary and Rheumatology, Tulane University, New Orleans, Louisiana; Department of Epidemiology and Health Promotion, New York University, New York, New York; Department of Medicine, Division of Rheumatology, Immunology and Allergy, Georgetown University, Washington, D.C., USA. hsuvm@rwjms.rutgers.edu.
Predictors of mortality and hospitalization in patients at risk for pulmonary hypertension (PH) include male sex, low DLCO, and pericardial effusions. Early intervention is crucial for those with systemic sclerosis (SSc) and PH risk factors.
Area of Science:
- Cardiology
- Pulmonology
- Rheumatology
Background:
- Pulmonary hypertension (PH) is a serious complication in systemic sclerosis (SSc).
- Identifying at-risk individuals is crucial for early intervention and improved outcomes.
- The PHAROS study investigates the natural history of PH in SSc patients.
Purpose of the Study:
- To identify predictors of mortality and cardiopulmonary hospitalizations in SSc patients at risk for PH.
- To evaluate baseline clinical measures as prognostic indicators.
- To inform clinical management strategies for SSc-associated PH.
Main Methods:
- Prospective cohort study (PHAROS) including 236 at-risk subjects.
- Defined at-risk criteria: echocardiogram sPAP > 40 mmHg, DLCO < 55%, or FVC/DLCO ratio > 1.6.
- Cox proportional hazards models used, adjusted for age, sex, race, and disease duration.
Main Results:
- 35 subjects developed PH during follow-up.
- Mortality predictors: male sex, low %DLCO, exercise oxygen desaturation, anemia, abnormal dyspnea, pericardial effusion.
- Cardiopulmonary hospitalization predictors: increased dyspnea, pericardial effusions; highest risk with DLCO < 50%.
Conclusions:
- Risk factors for poor outcomes in SSc patients at risk for PH mirror those with established SSc-PH.
- Male sex, low DLCO (<50%), exercise desaturation, and pericardial effusions are key risk factors.
- Recommend right heart catheterization and intervention for confirmed PH in this high-risk group.
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