1. An evidence-based strategy to screen for pulmonary arterial hypertension in systemic sclerosis
- Author
-
Janet E. Pope, David Langleben, Marvin J. Fritzler, Marie Hudson, T Semalulu, Thao Huynh, Mianbo Wang, Murray Baron, and Lawrence G. Rudski
- Subjects
medicine.medical_specialty ,Evidence-based practice ,Hypertension, Pulmonary ,Population ,Logistic regression ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,DLCO ,Diffusing capacity ,Internal medicine ,Humans ,Medicine ,Familial Primary Pulmonary Hypertension ,030212 general & internal medicine ,education ,030203 arthritis & rheumatology ,Pulmonary Arterial Hypertension ,education.field_of_study ,Scleroderma, Systemic ,business.industry ,Middle Aged ,Annual Screening ,Anesthesiology and Pain Medicine ,Case-Control Studies ,Cohort ,Population study ,Female ,business - Abstract
Background Clinical practice guidelines recommend screening all systemic sclerosis (SSc) patients for pulmonary arterial hypertension (PAH) with yearly echocardiograms. There is a paucity of evidence to support these guidelines. Research question Can a prediction model identify SSc patients with a very low probability of PAH and therefore not requiring annual screening echocardiogram? Study design and methods We performed a case-control study of 925 unselected SSc subjects nested in a multi-centered, longitudinal cohort. The probability of PAH for each subject was calculated using the results of multivariate logistic regression models. A cut-off was identified for the estimated probability of PAH below which no subject developed PAH (100% sensitivity). Results Study subjects were predominantly female (87.5%), with mean (SD) age 58.6 (11.7) years and disease duration of 18.2 (12.2) years. Thirty-seven subjects developed PAH during 5407.97 person-years of observation (incidence rate 0.68 per 100 person-years). Shortness of breath (SOB), diffusing capacity for carbon monoxide (DLCO) and NT-proBNP were independent predictors of PAH. All SSc-PAH cases had a probability of PAH of >1.1%. Subjects below this cut-off, none of whom had PAH, accounted for 46.2% of the study population. Interpretation A simple prediction model identified subjects at very low probability of PAH who could potentially forego annual screening echocardiogram. This represents almost half of SSc subjects in a general SSc population. This study, which is the first evidence-based study for the rational use of follow-up echocardiograms in an unselected SSc cohort, requires validation. The scoring system is freely available online at http://pahtool.ladydavis.ca.
- Published
- 2020
- Full Text
- View/download PDF