title: Exercise Doppler echocardiography identifies preclinic asymptomatic pulmonary hypertension in systemic sclerosis.
abstract: 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.
authors: Department of Medicine, Division of Rheumatology, University of Florence, Florence, Italy.
authors: Alberto
authors: A
authors: Pignone
authors: Fabio
authors: F
authors: Mori
authors: Francesco
authors: F
authors: Pieri
authors: Andrea
authors: A
authors: Oddo
authors: Gianna
authors: G
authors: Galeota
authors: Ginevra
authors: G
authors: Fiori
authors: Angela
authors: A
authors: Del Rosso
authors: Federico
authors: F
authors: Perfetto
authors: Aureliano
authors: A
authors: Becucci
authors: Riccardo
authors: R
authors: Livi
authors: Alessio
authors: A
authors: Tempestini
authors: Chiara
authors: C
authors: Benvenuti
authors: Leonardo
authors: L
authors: Gramigna
authors: Roberto
authors: R
authors: Fedi
authors: Sergio
authors: S
authors: Generini
authors: Martina
authors: M
authors: Minelli
authors: Marina
authors: M
authors: Cinelli
authors: Serena
authors: S
authors: Guiducci
authors: Chiara
authors: C
authors: Arcangeli
authors: Maria Letizia
authors: ML
authors: Conforti
authors: Pasquale
authors: P
authors: Bernardo
authors: Marco Matucci
authors: MM
authors: Cerinic
mesh_terms: 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*