Treatment of pulmonary arterial hypertension associated to systemic sclerosis

Purpose.-Pulmonary arterial hypertension (PAH) is one of the main causes of death in patients with systemic sclerosis (SSc), particularly in its limited forms. Current knowledge and keypoints.-Survival with conventional treatment associated with Epoprostenol is two times less in SSc patients than in idiopathic PAH. WHO recommendations (annually heart echoscreening) must be applied in all patients with SSc. Conventional therapy associates anticoagulation. to avoid excessive exertion, pregnancy, warm baths, no pressurised flights. Calcium-channel blockers give long term survival in patients with positive acute vasodilatator test with nitric oxide (NO) but these patients are very rare in SSc. Diuretics are very useful in treating right heart insufficiency. Randomized control trials in PAH have demonstrated the short term efficacy of IV epoprostenol, nebulized iloprost. oral beraprost and oral bosentan, a dual endothelin-1 receptor antagonist. Results in SSc are very limited except for IV epoprostenol. Long-term efficacy in terms of survival has been demonstrated in non randomized studies for TV epoprostenol and oral bosentan in patients with idiopathic and familial PAH. Atrial septostomy and lung transplantation could be an alternative treatment, even in SSc patients, in case of refractory evolution. Future prospects and projects.-Randomized control trials with sildenafil and selective endothelin-1 receptor antagonists are ongoing. (C) 2003 Elsevier SAS. Tons droits reserves.