874 A NOT-SO-RARE COMPLICATION OF CONNECTIVE TISSUE DISEASE

Abstract Pulmonary hypertension is a not-so-rare complication of connective tissue diseases and usually related with an inauspicious prognosis. We present the case 67-years-old woman hospitalized for progressive dyspnea. Two-dimensional echocardiography showed numerous features to pulmonary (PH) moderate Mitral Regurgitation. The right cardiac catheterization confirmed mixed PH (pre post capillary) without chest angio-TC evidence thromboembolism. Thanks optimized medical therapy (B-Blocker, ACE-i diuretics), there was significant clinical improvement documented at discharge. After 1 year, due recurrence dyspnea, she admitted our unit. Her examination some characteristics disease. Compared previous echocardiography, mild reduction MR documented. Chest CT scan revealed interstitial lung disease likely patient's underlying pathology. Immunological screening tests detected presence antinuclear (ANA) anti-topoisomerase (ATA) antibodies. Right heart worsening only pre-capillary component. On basis above findings, diagnosis CREST syndrome complicated made, patient started on oral combination macitentan riociguat. frequency this in disease, need different treatment approaches pre capillary discussed.