[CREST syndrome presenting as pulmonary hypertension]

The authors report the case of a 62-year-old patient complaining of recent onset of disabling breathlessness on exertion, and presenting clinical signs of previously undiagnosed scleroderma. Echocardiography revealed a diagnosis of precapillary pulmonary hypertension (74/14 mmHg) (PHT), with no pulmonary cause revealed by pulmonary ventilation-perfusion scintigraphy or by thoracic fine section computed tomography. The diagnosis of PHT in the context of circumscribed scleroderma was confirmed by x-rays of the hands, capillaroscopy, oesophageal investigations and positive anticentromere antinuclear antibodies. The clinical course was marked by rapid deterioration of the symptoms, requiring treatment with prostacyclin by continuous intravenous infusion. The appearance of PHT in a context of circumscribed scleroderma, usually a relatively benign disease, is a rare, late event, exceptionally revealing the disease, as in this case, and indicating a very unfavourable prognosis.