Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations

Christophe Meune, Jérôme Avouac, Paolo Airaò, Lorenzo Beretta, Philippe Dieudé, Karim Wahbi, Paola Caramaschi, Kiet Tiev, Susanna Cappelli, Elisabeth Diot, Alessandra Vacca, Jean Luc Cracowski, Jean Sibilia, André Kahan, Marco Matucci-Cerinic, Yannick Allanore

Research output: Contribution to journalArticle

50 Citations (Scopus)

Abstract

Objective To develop a score to estimate the risk of developing pulmonary hypertension (PH) in patients with systemic sclerosis (SSc). Methods We first examined the prevalence and characteristics of precapillary PH confirmed by right-heart catheterization in a cross-sectional (derivation) sample of 1,165 SSc patients, and we developed a risk prediction score (RPS) based on simple clinical observations associated with PH. We next prospectively tested the 3-year predictive power of the "Cochin RPS" in a separate (validation) sample of 443 patients presenting with PH-free SSc at baseline. Results In the derivation sample, age, forced vital capacity, and diffusing capacity for carbon monoxide/alveolar volume were independently associated with the presence of PH and were used to create the Cochin RPS. PH developed during followup in 20 patients in the validation sample. The area under the receiver operating characteristic curve of the Cochin RPS was 0.87 (95% confidence interval 0.79-0.95). With a cutoff value of 2.73, patients at risk of PH during followup could be identified with 89.5% sensitivity and 74.1% specificity. PH occurred in 0.6% of patients in the lowest 2 quintiles of the Cochin RPS, in 1.7% of patients in the third and fourth quintiles, and in 17.1% of patients in the highest quintile (P <0.0001 by log rank test). Patients in the highest quintile incurred a >35-fold higher risk of developing PH compared with patients in the 2 lowest quintiles (P = 0.001). Conclusion Using routine clinical observations, we developed a simple score that accurately predicted the risk of PH in SSc.

Original languageEnglish
Pages (from-to)2790-2796
Number of pages7
JournalArthritis and Rheumatism
Volume63
Issue number9
DOIs
Publication statusPublished - Sep 2011

Fingerprint

Systemic Scleroderma
Pulmonary Hypertension
Vital Capacity
Carbon Monoxide
Cardiac Catheterization
ROC Curve
Confidence Intervals
Sensitivity and Specificity

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Rheumatology
  • Pharmacology (medical)

Cite this

Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations. / Meune, Christophe; Avouac, Jérôme; Airaò, Paolo; Beretta, Lorenzo; Dieudé, Philippe; Wahbi, Karim; Caramaschi, Paola; Tiev, Kiet; Cappelli, Susanna; Diot, Elisabeth; Vacca, Alessandra; Cracowski, Jean Luc; Sibilia, Jean; Kahan, André; Matucci-Cerinic, Marco; Allanore, Yannick.

In: Arthritis and Rheumatism, Vol. 63, No. 9, 09.2011, p. 2790-2796.

Research output: Contribution to journalArticle

Meune, C, Avouac, J, Airaò, P, Beretta, L, Dieudé, P, Wahbi, K, Caramaschi, P, Tiev, K, Cappelli, S, Diot, E, Vacca, A, Cracowski, JL, Sibilia, J, Kahan, A, Matucci-Cerinic, M & Allanore, Y 2011, 'Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations', Arthritis and Rheumatism, vol. 63, no. 9, pp. 2790-2796. https://doi.org/10.1002/art.30432
Meune, Christophe ; Avouac, Jérôme ; Airaò, Paolo ; Beretta, Lorenzo ; Dieudé, Philippe ; Wahbi, Karim ; Caramaschi, Paola ; Tiev, Kiet ; Cappelli, Susanna ; Diot, Elisabeth ; Vacca, Alessandra ; Cracowski, Jean Luc ; Sibilia, Jean ; Kahan, André ; Matucci-Cerinic, Marco ; Allanore, Yannick. / Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations. In: Arthritis and Rheumatism. 2011 ; Vol. 63, No. 9. pp. 2790-2796.
@article{4eb2560867e74ddbbc8b80a1f77f0bd7,
title = "Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations",
abstract = "Objective To develop a score to estimate the risk of developing pulmonary hypertension (PH) in patients with systemic sclerosis (SSc). Methods We first examined the prevalence and characteristics of precapillary PH confirmed by right-heart catheterization in a cross-sectional (derivation) sample of 1,165 SSc patients, and we developed a risk prediction score (RPS) based on simple clinical observations associated with PH. We next prospectively tested the 3-year predictive power of the {"}Cochin RPS{"} in a separate (validation) sample of 443 patients presenting with PH-free SSc at baseline. Results In the derivation sample, age, forced vital capacity, and diffusing capacity for carbon monoxide/alveolar volume were independently associated with the presence of PH and were used to create the Cochin RPS. PH developed during followup in 20 patients in the validation sample. The area under the receiver operating characteristic curve of the Cochin RPS was 0.87 (95{\%} confidence interval 0.79-0.95). With a cutoff value of 2.73, patients at risk of PH during followup could be identified with 89.5{\%} sensitivity and 74.1{\%} specificity. PH occurred in 0.6{\%} of patients in the lowest 2 quintiles of the Cochin RPS, in 1.7{\%} of patients in the third and fourth quintiles, and in 17.1{\%} of patients in the highest quintile (P <0.0001 by log rank test). Patients in the highest quintile incurred a >35-fold higher risk of developing PH compared with patients in the 2 lowest quintiles (P = 0.001). Conclusion Using routine clinical observations, we developed a simple score that accurately predicted the risk of PH in SSc.",
author = "Christophe Meune and J{\'e}r{\^o}me Avouac and Paolo Aira{\`o} and Lorenzo Beretta and Philippe Dieud{\'e} and Karim Wahbi and Paola Caramaschi and Kiet Tiev and Susanna Cappelli and Elisabeth Diot and Alessandra Vacca and Cracowski, {Jean Luc} and Jean Sibilia and Andr{\'e} Kahan and Marco Matucci-Cerinic and Yannick Allanore",
year = "2011",
month = "9",
doi = "10.1002/art.30432",
language = "English",
volume = "63",
pages = "2790--2796",
journal = "Arthritis care and research : the official journal of the Arthritis Health Professions Association",
issn = "0893-7524",
publisher = "John Wiley and Sons Inc.",
number = "9",

}

TY - JOUR

T1 - Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations

AU - Meune, Christophe

AU - Avouac, Jérôme

AU - Airaò, Paolo

AU - Beretta, Lorenzo

AU - Dieudé, Philippe

AU - Wahbi, Karim

AU - Caramaschi, Paola

AU - Tiev, Kiet

AU - Cappelli, Susanna

AU - Diot, Elisabeth

AU - Vacca, Alessandra

AU - Cracowski, Jean Luc

AU - Sibilia, Jean

AU - Kahan, André

AU - Matucci-Cerinic, Marco

AU - Allanore, Yannick

PY - 2011/9

Y1 - 2011/9

N2 - Objective To develop a score to estimate the risk of developing pulmonary hypertension (PH) in patients with systemic sclerosis (SSc). Methods We first examined the prevalence and characteristics of precapillary PH confirmed by right-heart catheterization in a cross-sectional (derivation) sample of 1,165 SSc patients, and we developed a risk prediction score (RPS) based on simple clinical observations associated with PH. We next prospectively tested the 3-year predictive power of the "Cochin RPS" in a separate (validation) sample of 443 patients presenting with PH-free SSc at baseline. Results In the derivation sample, age, forced vital capacity, and diffusing capacity for carbon monoxide/alveolar volume were independently associated with the presence of PH and were used to create the Cochin RPS. PH developed during followup in 20 patients in the validation sample. The area under the receiver operating characteristic curve of the Cochin RPS was 0.87 (95% confidence interval 0.79-0.95). With a cutoff value of 2.73, patients at risk of PH during followup could be identified with 89.5% sensitivity and 74.1% specificity. PH occurred in 0.6% of patients in the lowest 2 quintiles of the Cochin RPS, in 1.7% of patients in the third and fourth quintiles, and in 17.1% of patients in the highest quintile (P <0.0001 by log rank test). Patients in the highest quintile incurred a >35-fold higher risk of developing PH compared with patients in the 2 lowest quintiles (P = 0.001). Conclusion Using routine clinical observations, we developed a simple score that accurately predicted the risk of PH in SSc.

AB - Objective To develop a score to estimate the risk of developing pulmonary hypertension (PH) in patients with systemic sclerosis (SSc). Methods We first examined the prevalence and characteristics of precapillary PH confirmed by right-heart catheterization in a cross-sectional (derivation) sample of 1,165 SSc patients, and we developed a risk prediction score (RPS) based on simple clinical observations associated with PH. We next prospectively tested the 3-year predictive power of the "Cochin RPS" in a separate (validation) sample of 443 patients presenting with PH-free SSc at baseline. Results In the derivation sample, age, forced vital capacity, and diffusing capacity for carbon monoxide/alveolar volume were independently associated with the presence of PH and were used to create the Cochin RPS. PH developed during followup in 20 patients in the validation sample. The area under the receiver operating characteristic curve of the Cochin RPS was 0.87 (95% confidence interval 0.79-0.95). With a cutoff value of 2.73, patients at risk of PH during followup could be identified with 89.5% sensitivity and 74.1% specificity. PH occurred in 0.6% of patients in the lowest 2 quintiles of the Cochin RPS, in 1.7% of patients in the third and fourth quintiles, and in 17.1% of patients in the highest quintile (P <0.0001 by log rank test). Patients in the highest quintile incurred a >35-fold higher risk of developing PH compared with patients in the 2 lowest quintiles (P = 0.001). Conclusion Using routine clinical observations, we developed a simple score that accurately predicted the risk of PH in SSc.

UR - http://www.scopus.com/inward/record.url?scp=80052339811&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=80052339811&partnerID=8YFLogxK

U2 - 10.1002/art.30432

DO - 10.1002/art.30432

M3 - Article

VL - 63

SP - 2790

EP - 2796

JO - Arthritis care and research : the official journal of the Arthritis Health Professions Association

JF - Arthritis care and research : the official journal of the Arthritis Health Professions Association

SN - 0893-7524

IS - 9

ER -