Pulmonary arterial hypertension in rheumatological practice: insidious beginning – severe consequences. The importance of early detection

Pulmonary arterial hypertension (PAH) is a chronic, progressive disease leading to death. It is important in rheumatological practice as a leading cause of mortality in systemic sclerosis. PAH can be a complication of other connective tissue diseases. During the last decade there has been substantial progress in understanding of PAH pathogenesis, leading to the introduction of new therapies. However, the response to modern therapies in scleroderma-related PAH seems to be less promising compared to other forms of PAH. One of the reasons may be diagnosis delay. In this context introduction of effective screening procedures seems to be crucial. Nowadays, yearly echocardiography is recommended in all patients with systemic sclerosis and symptomatic patients with other connective tissue diseases (CTD). Investigations to optimize the screening algorithm with new echocardiographic techniques and use of biomarkers (NT-proBNP) are under way. The suspicion of PAH has to be confirmed by right heart catheterization. The review discusses current strategy to diagnose and assess CTD-related PAH in everyday practice.