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Rheumatology 2006 45(Supplement 3):iii49-iii51; doi:10.1093/rheumatology/kel283
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Vasoactive therapies in systemic sclerosis

G. Riemekasten and C. Sunderkötter1

Department of Medicine (Rheumatology and Clinical Immunology), Charité University Hospital, Humboldt-University of Berlin and 1Department of Dermatology and Venerology, University Hospital Münster, Germany.

Correspondence to: Gabriela Riemekasten, MD. Department of Rheumatology and Clinical Immunology, Charité University Hospital, Schumannstr. 20/21, D-10117 Berlin, Germany. E-mail: gabriela.riemekasten{at}charite.de

In systemic sclerosis (SSc), vasculopathy is a central mechanism and is a major initial event in the process of sclerosis and causing different complications such as Raynaud's phenomenon, ulcer(s) or pulmonary hypertension, the latter being life threatening. Therefore, vasoactive therapies are important when taking care of patients with SSc. However, as treatment has been difficult, numerous therapeutic modalities have been suggested. Until now, the interpretation of most studies is limited due to the heterogeneity of patient groups, the low number of patients, the short duration of the treatments and, possibly, further pathogenic mechanisms such as autoimmunity.

Several drugs are now available with effects on vasculopathy and, furthermore, on specific pathogenic mechanisms in SSc. Prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors have potential effects on fibrosis, inflammation and endothelial cells, suggesting a disease-modifying capacity in systemic sclerosis.

This review summarizes evidence-based therapy recommendations.


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