Treatments of progressive systemic sclerosis

Progressive Systemic Sclerosis (PSS) is still an incurable disease but ther e are treatments for it, and the list of proposed treatments is long. The methodology of trials concerning PSS is complex, due to the low prevale nce of the disease and therefore its financial interest for pharmaceutical companies the lack of simple end points for efficacy, and the large number of clinical expressions with various prognoses. These causes explain why mo st open studies are fiercely positive, and why controlled studies are so ra re. The progress made during the last 10 years concern 1) the diagnosis, which is now made earlier due to capillaroscopy and antibody assays, especially o f anticentromere antibodies, 2) better knowledge of the prognosis, due to t he classification of PSS into limited and the diffuse forms which have diff erent prognoses, and 3) the recognition of a serious complication of the di sease, ic. pulmonary hypertension, which can now be detected by noninvasive methods. All these improvements will also improve the methodology of futur e trials of drugs for treating PSS. In this ocean of uncertainties, some treatments have a valid background, an d 3 visceral locations of PSS can be efficiently treated: renal involvement , with angiotensin-converting enzyme inhibitors, respiratory involvement, w ith D-penicillamine, and pulmonary hypertension, with prostacyclin derivati ves. Corticosteroids are suspected to increase the risk of renal complicati ons. Calcium blockers are considered a useful symptomatic treatment of the associated Raynaud's phenomenon and of the risk of digital necrosis, and ma y also constitute a treatment of PSS it self. A recent trial conducted by the French Microcirculation Society and its acr osyndrome Study Group considered the effects of an oral derivative of prost acyclin. Beneficial effects were: a reduction of the risk of digital necros es, improved overall wellbeing, less necessity for hospitalizations. fewer giant capillaries, and a dramatic fall in the level of von-Willebrand facto r.