[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.
Hunzelmann
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Klinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln, Kerpener Strasse 62, 50937 Köln. nico.hunzelmann@uni-koeln.de
Riemekasten
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G
D002407:Catheterization, Swan-Ganz; D003240:Connective Tissue Diseases / Q000150:complications* / Q000503:physiopathology; D004452:Echocardiography; D005858:Germany / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000188:drug therapy / Q000209:etiology; D011669:Pulmonary Wedge Pressure; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D012955:Societies, Medical / Q000458:organization & administration
D007166:Immunosuppressive Agents