An evidence-based strategy to screen for pulmonary arterial hypertension in systemic sclerosis.


Journal

Seminars in arthritis and rheumatism
ISSN: 1532-866X
Titre abrégé: Semin Arthritis Rheum
Pays: United States
ID NLM: 1306053

Informations de publication

Date de publication:
12 2020
Historique:
received: 18 10 2019
revised: 20 02 2020
accepted: 25 02 2020
pubmed: 5 4 2020
medline: 30 9 2021
entrez: 5 4 2020
Statut: ppublish

Résumé

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. Can a prediction model identify SSc patients with a very low probability of PAH and therefore not requiring annual screening echocardiogram? 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). 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. 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.

Sections du résumé

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.

Identifiants

pubmed: 32245697
pii: S0049-0172(20)30066-4
doi: 10.1016/j.semarthrit.2020.02.013
pii:
doi:

Types de publication

Journal Article Multicenter Study Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1421-1427

Informations de copyright

Copyright © 2020 Elsevier Inc. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest All authors declare no conflicts of interest.

Auteurs

T Semalulu (T)

Department of Medicine, McMaster University, Canada.

L Rudski (L)

Department of Medicine, McGill University, Montreal, Canada; Division of Cardiology, Jewish General Hospital, Montreal, Canada.

T Huynh (T)

Department of Medicine, McGill University, Montreal, Canada; Division of Cardiology, McGill University Health Centre, Montreal, Canada.

D Langleben (D)

Department of Medicine, McGill University, Montreal, Canada; Division of Cardiology, Jewish General Hospital, Montreal, Canada; Lady Davis Institute for Medical Research, Montreal, Canada.

M Wang (M)

Lady Davis Institute for Medical Research, Montreal, Canada.

M J Fritzler (MJ)

Cumming School of Medicine, University of Calgary, Calgary, Canada.

J Pope (J)

St. Joseph's Healthcare, London, Canada.

M Baron (M)

Department of Medicine, McGill University, Montreal, Canada; Division of Rheumatology, Jewish General Hospital, Room A-725, 3755 Côte Sainte-Catherine Road, Montreal, Quebec H3T 1E2, Canada.

M Hudson (M)

Department of Medicine, McGill University, Montreal, Canada; Lady Davis Institute for Medical Research, Montreal, Canada; Division of Rheumatology, Jewish General Hospital, Room A-725, 3755 Côte Sainte-Catherine Road, Montreal, Quebec H3T 1E2, Canada. Electronic address: marie.hudson@mcgill.ca.

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