PT  - JOURNAL ARTICLE
AU  - Hoeper, M.M.
TI  - Pulmonary hypertension in collagen vascular disease
AID  - 10.1183/09031936.02.01512001
DP  - 2002 Mar 01
TA  - European Respiratory Journal
PG  - 571--576
VI  - 19
IP  - 3
4099  - https://publications.ersnet.org//content/19/3/571.short
4100  - https://publications.ersnet.org//content/19/3/571.full
SO  - Eur Respir J2002 Mar 01; 19
AB  - Pulmonary hypertension is a serious but often overlooked complication in collagen vascular disease. The understanding of the development of pulmonary hypertension has increased substantially during the last years. Abnormal proliferation of pulmonary vascular cells is now being regarded as a predominant process leading to pulmonary vascular obliteration. Medical therapy focuses on prostacyclin treatment, which has been shown to improve exercise capacity and haemodynamic variables in patients with several collagen vascular diseases and pulmonary arterial hypertension. Continuous intravenous prostacyclin remains the standard treatment of associated pulmonary hypertension but less invasive alternatives such as subcutaneous treprostinil, oral beraprost or aerosolized iloprost, as well as, novel substances such as endothelin receptor antagonists may be appropriate for selected patients.