Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases
Anna Smukowska-Gorynia,
Weronika Gościniak,
Patrycja Woźniak
et al.
Abstract:Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to diverse groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) secondary PH due to lung disease and/or hypoxia and (4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis. Therefore,… Show more
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