Pulmonary hypertension screening in systemic sclerosis: apropos 67 patients.

Purpose. - Pulmonary hypertension is a severe complication of systemic scle rosis and has emerged as a major cause of morbidity and mortality in this c ondition. Treatment is all the more efficient as pulmonary hypertension is early diagnosed, A good knowledge of the clinical, biological and functiona l features of pulmonary hypertension in systemic sclerosis is therefore nec essary to suspect and to diagnose pulmonary hypertension as early as possib le. Methods. - Sixty seven patients with systemic sclerosis were retrospectivel y studied. We compared clinical, immunological, functional (spirometry) and morphological (pulmonary fibrosis) features according to the presence (n = 25) and the characteristic of pulmonary hypertension (isolated or secondar y) or the absence (n = 42) of pulmonary hypertension, assessed by Doppler e chocardiography. Results. - CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal in volvement, sclerodactyly and telangiectasia) was more frequent in patients with isolated pulmonary hypertension than in patients without PH (72.7% vs 28.5%, P < 0.05; odds-ratio [OR] = 6.6) and dyspnea was more severe (P < 0. 001; OR = 11.4). The age at time of pulmonary hypertension diagnosis was hi gher in patients with secondary pulmonary hypertension than in patients wit h isolated form (median: 62.5 years (range: 32-35) vs 53 years (range: 37-8 5), P < 0.05). Patients with isolated pulmonary hypertension had anticardio lipin antibodies more frequently than patients without pulmonary hypertensi on (72.7% vs 35.7%, P < 0.05). Isolated reduction of diffusing capacity was preferentially observed among patients with isolated pulmonary hypertensio n than among those without pulmonary hypertension, A linear relation betwee n systolic pulmonary artery pressure values and diffusing capacity values ( r = 0.72, P < 0.01) was found. Isolated reduction of diffusing capacity was more frequent in patients with isolated pulmonary hypertension than in pat ients without pulmonary hypertension (63.6% vs 14.3%, P < 0.001; OR = 10.5) . Conclusion. - The severity of pulmonary hypertension in systemic sclerosis justifies a systematic screening by Doppler echocardiography and diffusing capacity measurement. Our results allow us to better define the characteris tics of sclerodermic patients with isolated or secondary pulmonary hyperten sion. The search for pulmonary hypertension should be repeated with time an d clinicians should be particularly vigilant in the case of a patient prese nting these characteristics. (C) 2001 Editions scientifiques et medicales E lsevier SAS.