[Pulmonary arterial hypertension in collagen disease: experiences of the German Network for Systemic Scleroderma].

The German Network for Systemic Scleroderma (Deutsches Netzwerk Für Systemische Sklerodermie [DNSS]) has as its aim to improve and standardize the diagnosis and treatment of this rare disease. 14.6 % of patients in the DNSS register have been shown to have pulmonary hypertension (PHT). Comparable international data range from 12 % to 26.7 %. The DNSS recommends annual echocardiography for patients with scleroderma and right-heart catheterization if the systolic pulmonary arterial pressure is higher than 35 mmHg. Aggressive immunosuppressive treatment--in the first instance with cyclophosphamide--should be given only if there is also progressive fibrosis. Drugs that lower the level of PHT also have a favourable effect on other signs of scleroderma.