Pulmonary involvement in systemic sclerosis. Part 2: isolated pulmonary arterial hypertension, lung cancer, alveolar hemorrhage.

Introduction. - Pulmonary interstitial fibrosis is the most frequent cause of lung disease in systemic sclerosis. However, other pulmonary complicatio ns exist, including lung cancer, alveolar hemorrhage, and in particular iso lated pulmonary arterial hypertension, which is still considered the bete n oire as regards this disease. Current knowledge and key points. - The prevalence of pulmonary arterial hy pertension has been reported to range from 5 to 60% in cases of systemic sc lerosis; isolated pulmonary arterial hypertension has been principally obse rved ill subjects with a ten-year history of limited forms of the disease. As the patient remains asymptomatic for a long period, with nonspecific res piratory clinical manifestations, the diagnosis is made at a much late,- st age ii? the course of the disease. The diagnostic method of choice is echoc ardiography-doppler, which should be performed during the preliminary inves tigation, and at follow-up. The prognosis is poor, and patient survival rat e at 2 years after onset of symptoms amounts to 40%. To date, no curative t herapy So,- pulmonary arterial hypertension has yet been found. Future prospects and projects. - A knowledge of the mechanisms involved ill the development of isolated pulmonary arterial hypertension is essential t o the determination of new and relevant therapeutic strategies. Vasodilator y treatment, notably calcium channel blockers, prostacyclin and analogs suc h as iloprost, may ne effective at an early stage of the disease before the appearance of permanent vascular damage. (C) 1999 Editions scientifiques e t medicales Elsevier SAS.