Exercise Doppler echocardiography identifies preclinic asymptomatic pulmonary hypertension in systemic sclerosis.
In systemic sclerosis (SSc), the involvement of the interstitium or vascular system of the lung may lead to pulmonary arterial hypertension (PAH). PAH is often asymptomatic or oligosymptomatic in early SSc and, when it becomes symptomatic, pulmonary vascular system is already damaged. Exercise echocardiography (ex-echo), measuring pulmonary artery pressure (PAP) during exercise and allowing to differentiate physiologic from altered PAP responses, may identify subclinical PAH. Our aims were (a) to evaluate by ex-echo the change of PAP in patients with SSc without lung involvement; and (b) to correlate PAP during exercise (ex-PAP) values to clinical and biohumoral parameters of PAH. Twenty-seven patients with limited SSc (ISSc) without interstitial lung involvement were studied. Patients underwent rest and exercise two-dimensional and Doppler echocardiography by supine cycloergometer. Systolic PAP was calculated using the maximum systolic velocity of the tricuspid regurgitant jet at rest and during exercise values of systolic PAP exceeding 40 mmHg at ex-echo were considered as abnormal, and biohumoral markers potentially related to PAH were assessed. Eighteen of 27 SSc patients presented an ex-PAP > 40 mmHg, while in 9 of 27 patients ex-PAP values remained < 40 mmHg (48.8 +/- 4.5 mmHg versus 36.2 +/- 3.1 mmHg; P < 0.001). Other echocardiographic and ergometric parameters, clinical tests, and biohumoral markers were not different in the two groups. Ex-PAP significantly correlated with D-dimer (P = 0.0125; r2 = 0.2029). Ex-echo identifies a cluster of SSc patients with subclinical PAH that may develop PAH. This group should be followed up and may be considered for specific therapies to prevent disease evolution.
Pignone
Alberto
A
Department of Medicine, Division of Rheumatology, University of Florence, Florence, Italy.
Mori
Fabio
F
Pieri
Francesco
F
Oddo
Andrea
A
Galeota
Gianna
G
Fiori
Ginevra
G
Del Rosso
Angela
A
Perfetto
Federico
F
Becucci
Aureliano
A
Livi
Riccardo
R
Tempestini
Alessio
A
Benvenuti
Chiara
C
Gramigna
Leonardo
L
Fedi
Roberto
R
Generini
Sergio
S
Minelli
Martina
M
Cinelli
Marina
M
Guiducci
Serena
S
Arcangeli
Chiara
C
Conforti
Maria Letizia
ML
Bernardo
Pasquale
P
Cerinic
Marco Matucci
MM
D015150:Echocardiography, Doppler; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications*