Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies.
OBJECTIVE
To measure the prevalence of different types of pulmonary hypertension (PH) and to identify patients with systemic sclerosis (SSc) at highest risk in a multicenter European sample, with a metaanalysis of relevant studies.


METHODS
Consecutive patients with SSc recruited at 11 French and Italian centers underwent detailed evaluations, including Doppler echocardiography, chest computed tomography, pulmonary function tests, and right-heart catheterization (RHC), to detect the presence and causes of PH. A metaanalysis was performed, including data from 4 other studies.


RESULTS
Among 206 patients in whom it was suspected, PH was confirmed by RHC in 83 patients (7%). Precapillary PH was found in 64 patients (5%), of whom 42 had pulmonary arterial hypertension (PAH) and 22 had PH secondary to interstitial lung disease (ILD). RHC identified 17 patients (1%) with postcapillary PH secondary to left-heart disease. Patients with DLCO/alveolar volume < 70% were more likely to have precapillary PH (87.5% vs 42%; p < 0.0001). Precapillary and postcapillary PH were associated with advanced age (68 ± 14 vs 59 ± 12 yrs, p < 0.0001, and 74 ± 16 vs 61.5 ± 10 yrs, p < 0.0001, respectively). The metaanalysis of 3818 patients showed a prevalence of precapillary PH of 9% (95% CI 6%-12%) and identified advanced age, longer disease duration, and limited cutaneous disease subset as risk factors for this condition.


CONCLUSIONS
The prevalence of precapillary PH in our multicenter study of SSc was 5%, and in the metaanalysis 9%. Our observations support use of RHC to confirm the presence of precapillary PH suspected by noninvasive testing. We also identified patients at high risk who should be carefully monitored.
Avouac
Jérôme
J
Department of Rheumatology A, Paris Descartes University, Cochin Hospital, APHP, Paris, France.
Airò
Paolo
P
Meune
Christophe
C
Beretta
Lorenzo
L
Dieude
Philippe
P
Caramaschi
Paola
P
Tiev
Kiet
K
Cappelli
Susanna
S
Diot
Elisabeth
E
Vacca
Alessandra
A
Cracowski
Jean-Luc
JL
Sibilia
Jean
J
Kahan
André
A
Matucci-Cerinic
Marco
M
Allanore
Yannick
Y
D000367:Age Factors; D000368:Aged; D006328:Cardiac Catheterization; D015897:Comorbidity; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology*; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012595:Scleroderma, Systemic / Q000453:epidemiology*; D012720:Severity of Illness Index; D044465:White People