Prevalence of pulmonary hypertension in systemic sclerosis

Objective To assess the prevalence of pulmonary arterial hypertension (PAH) in patients with the diagnosis of systemic sclerosis (SSc) followed at a tertiary university service. Material and Methods Fifty-seven patients with SSc were studied by clinical assessment directed at the cardiopulmonary system, pulmonary function tests and Doppler echocardiography (ECHO). The following criteria were considered for the diagnosis of PAH: pulmonary artery systolic pressure (PASP) >= 40 mmHg and/or the presence of other direct and indirect signs of PAH detected upon ECHO. Results Sixteen patients (28%) were diagnosed with PAH upon ECHO, 13 based on PASP >= 40 mmHg and 3 based on direct and indirect signs of PAH; 8 patients had isolated PAH and 8 had PAH secondary to pulmonary fibrosis. Nine patients showed signs suggestive of cor pulmonale upon ECHO; among these patients, 6 had pressure recordings 40 mmHg and 3 had a PSAP between 35 and 40 mmHg; one patient was asymptomatic and 8 showed signs suggestive of PAH upon clinical examination. Among the clinical and laboratory variables studied, a correlation was only observed between PAH and an elevated erythrocyte sedimentation rate (p = 0.004). Conclusions The prevalence of PAH associated with SSc observed in this study was similar to those reported in the literature. However the cut-off of PSAP measured by ECHO and used for the diagnosis of PAH associated with SSc needs to be revised.