[Primary pulmonary hypertension: a rare but often fatal complication of Crest syndrome].

In a retrospective study of 194 systemic sclerosis, we selected 37 Crest syndrome patients. Among these 37 patients, five presented a primary pulmonary hypertension. For three of these patients, the evolution of this primary pulmonary hypertension was rapidly fatal, one died of another cause. One died without certitude that death is imputable to HTAP. Diagnosis and management of the evolution of primary pulmonary hypertension may be easily assessed by doppler-echocardiography.