A Clinical Decision Tool for Risk Stratifying Patients with Systemic Sclerosis-Related Pulmonary Hypertension

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

doi:10.1007/s00408-023-00646-2

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A Clinical Decision Tool for Risk Stratifying Patients with Systemic Sclerosis-Related Pulmonary Hypertension

Journal article

Peer reviewed

A Clinical Decision Tool for Risk Stratifying Patients with Systemic Sclerosis-Related Pulmonary Hypertension

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 and Elizabeth S Klings

Lung, Vol.201(6), pp.565-569

12/01/2023

DOI: 10.1007/s00408-023-00646-2

PMCID: PMC11037922

PMID: 37957388

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https://pmc.ncbi.nlm.nih.gov/articles/PMC11037922/pdf/nihms-1984760.pdfView

Open Access

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.

Cardiac Catheterization - adverse effects

Echocardiography - adverse effects

Humans

Hypertension, Pulmonary - diagnosis

Hypertension, Pulmonary - etiology

Retrospective Studies

Scleroderma, Systemic - complications

Scleroderma, Systemic - diagnosis

Details

Title: Subtitle: A Clinical Decision Tool for Risk Stratifying Patients with Systemic Sclerosis-Related Pulmonary Hypertension
Creators: Justin K Lui - Boston University
Kari R Gillmeyer - Boston University
Ruchika A Sangani - Boston University
Robert J Smyth - Boston University
Deepa M Gopal - Boston University
Marcin A Trojanowski - Boston University
Andreea M Bujor - Boston University
Renda Soylemez Wiener - VA Boston Healthcare System
Michael P LaValley - Boston University
Elizabeth S Klings - Boston University
Resource Type: Journal article
Publication Details: Lung, Vol.201(6), pp.565-569
DOI: 10.1007/s00408-023-00646-2
PMID: 37957388
PMCID: PMC11037922
NLM abbreviation: Lung
ISSN: 0341-2040
eISSN: 1432-1750
Grant note: 1UL1TR001430 / NCATS NIH HHS P30 AR072571 / NIAMS NIH HHS R01 HL155955 / NHLBI NIH HHS 1UG3 HL143192-01A1 / NHLBI NIH HHS 2021261 / Doris Duke Charitable Foundation CCCR P30 AR072571 / NIAMS NIH HHS 1R01HL155955-01A1 / NHLBI NIH HHS UL1 TR001430 / NCATS NIH HHS UG3 HL143192 / NHLBI NIH HHS U1EMC27864-08-00 / HRSA HHS
Language: English
Date published: 12/01/2023
Academic Unit: Pulmonary, Critical Care, and Occupational Medicine; Internal Medicine
Record Identifier: 9984695679902771

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