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1: Dtsch Med Wochenschr. 2006 Dec 8;131(49 Suppl 9):S325-7.Click here to read Links
Comment in:
Dtsch Med Wochenschr. 2007 Feb 16;132(7):337; author reply 338.

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

[Article in German]

Klinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln, Kerpener Strasse 62, 50937 Köln. nico.hunzelmann@uni-koeln.de

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.

PMID: 17139598 [PubMed - indexed for MEDLINE]

Patient Drug Information

  • Cyclophosphamide (Cytoxan® , Neosar® )

    Your doctor has ordered the drug cyclophosphamide to help treat your illness. The drug can be taken by mouth in tablet form or be given by injection into a vein.