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.
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U2 - 10.1002/art.30432
DO - 10.1002/art.30432
M3 - Article
C2 - 21547892
AN - SCOPUS:80052339811
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 -