OBJECTIVE: To assess the prevalence of pulmonary arterial hypertension (PAH) in patients with diagnosis of systemic sclerosis (SSc) evaluated at a university tertiary service. METHODS: Fifty-seven SSc patients attending the Rheumatology outpatient clinic of HC-UFMG were studied by clinical assessment addressed to cardiopulmonary system, pulmonary function tests and Doppler echocardiography (ECO). The following criteria were considered for the diagnosis of PAH: pulmonary arterial systolic pressure (PASP) = 40mmHg and/or the presence of direct or indirect signs of PAH found during the ECO. RESULTS: Sixteen patients (28%) were found to have the diagnosis of PAH at ECO, 13 with PASP = 40mmHg and 3 with direct and indirect signs of PAH; 8 patients had isolated PAH and 8, PAH secondary to pulmonary fibrosis. Nine patients had suggestive signs of cor pulmonale at ECO; 6 of these patients presented PASP = 40mmHg and 3 PASP between 35 and 40mmHg; 1 of them was not symptomatic and 8 had clinical exam suggestive of PAH. Regarding the clinical and laboratory variables studied, it was observed statistical association between PAH and elevated erythrocyte sedimentation rate (ESR) (p = 0,004). CONCLUSIONS: There was a prevalence of PAH in SSc similar to that observed in the literature, in spite of limitations in this comparison. The assessment of PAH by ECO is accessible and practical; however, it is necessary to review the cut off of the PASP levels considered for the diagnosis of PAH in SSc, especially when considering an early diagnosis.
systenic sclerosis; pulmonary arterial hypertension; doppler echocardiography
