Published in:
13-11-2023 | Pulmonary Hypertension | PULMONARY HYPERTENSION
A Clinical Decision Tool for Risk Stratifying Patients with Systemic Sclerosis-Related Pulmonary Hypertension
Authors:
Justin K. Lui, Kari R. Gillmeyer, Ruchika A. Sangani, Robert J. Smyth, Deepa M. Gopal, Marcin A. Trojanowski, Andreea M. Bujor, Renda Soylemez Wiener, Michael P. LaValley, Elizabeth S. Klings
Published in:
Lung
|
Issue 6/2023
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Abstract
We devised a scoring system to identify patients with systemic sclerosis (SSc) at risk for pulmonary hypertension (PH) and predict all-cause mortality. Using 7 variables obtained via pulmonary function testing, echocardiography, and computed tomographic chest imaging, we applied the score to a retrospective cohort of 117 patients with SSc. There were 60 (51.3%) who were diagnosed with PH by right heart catheterization. Using a scoring threshold ≥ 0, our decision tool predicted PH with a sensitivity, specificity, and accuracy of 0.87 (95% CI 0.75, 0.94), 0.74 (95% CI 0.60, 0.84), and 0.80 (95% CI 0.72, 0.87), respectively. When adjusted for age at PH diagnosis, sex, and receipt of pulmonary arterial vasodilators, each one-point score increase was associated with an adjusted HR of 1.19 (95% CI 1.05, 1.34) for all-cause mortality. With further validation in external cohorts, our simplified clinical decision tool may better streamline earlier detection of PH in SSc.