[Pulmonary arterial hypertension in collagenoses]

Pulmonary arterial hypertension is a rare disease of small pulmonary arteries of unknown origin characterised by endothelial dysfunction and cellular proliferation throughout all vessel layers, resulting in progressively elevated pulmonary arterial resistance with increasing right heart strain and finally right heart failure. The condition may develop in connective tissue diseases with variable frequency leading to a substantial worsening of prognosis. However, the spectrum of therapeutic options has broadened significantly in recent years. Several compounds have gained approval that act mainly as pulmonary vasodilators. Further drugs are under investigation, some of which target pulmonary vascular remodeling. Echocardiography remains the primary examination for disease detection. To classify pulmonary hypertension definite hemodynamic evaluation by means of right heart catheterisation and a thorough differential diagnosis are essential to provide the basis for further treatment. For differential therapy and assessment of follow-up profound knowledge is required, pointing to the need for close cooperation with specialised centres.