| Abstract | Systemic sclerosis (scleroderma) is considered as the most severe connective tissue disease. It is characterized by an abnormal immune activation, a vasculopathy and a fibrosis of the skin and of multiple internal organs. Numerous progress in the understanding of the pathogenesis with identification of key molecules have permit to introduce novel treatments that improve the management of some aspects of the disease. ACE inhibitors are effective in resolving renal crisis. Cyclophosphamide is useful for treatment of fibrosing alveolitis. Prostaglandins, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors permit to improve the treatment of the vascular complications (digital ulcerations, pulmonary arterial hypertension) of scleroderma. |
| Authors | J P Zuber, C Chizzolini, A Leimgruber, P A Bart, F Spertini
(Affiliation: Service d'immunologie et d'allergie Département de médecine CHUV, BH 18/707. jean-philippe.zuber at chuv.ch)
|
| Journal | Revue médicale suisse
(Rev Med Suisse)
Vol. 2
Issue 62
Pg. 1058, 1060-6
(Apr 19 2006)
ISSN: 1660-9379 Switzerland |
| Vernacular Title | Mécanismes pathogéniques de la sclérodermie et leurs conséquences thérapeutiques. 2e partie: traitement. |
| PMID | 16711151
(Publication Type: English Abstract, Journal Article, Review)
|
| Topics |
- Fingers
- Humans
- Hypertension, Pulmonary
(etiology)
- Raynaud Disease
(etiology)
- Scleroderma, Systemic
(complications, therapy)
- Skin Ulcer
(etiology)
|