Pulmonary arterial hypertension in systemic sclerosis: diagnostic pathway and therapeutic approach

Abstract

Laboratory tests included high erythrocyte sedimentation rate (145 mm/1st h), C reactive protein (2530 mg/l, normal 9/l), decreased arterial oxygen and carbon dioxide pressures. A chest x ray examination showed cardiomegaly, dilatation of the main pulmonary artery (fig 1), centralisation of pulmonary vascular flow, and reticular opacities in the lower parts of the lungs. Electrocardiography (ECG) at admission did not show any significant abnormalities. Doppler echocardiography disclosed a pulmonary artery systolic pressure (PASP) of 86 mm Hg, suggesting a severe pulmonary artery hypertension (PAH). Pulmonary function tests showed a reduction of forced vital capacity (FVC, 45% of predicted), and a high resolution chest computed tomography (CT) scan showed interstitial lung disease (ground glass and interstitial fibrosis) (fig 1). Furthermore, the patient developed pulmonary embolism, confirmed with perfusion scintigraphy (fig 1), with an increase in pulmonary pressure up to 115 mm Hg. Anticardiolipin antibodies were found to be positive.