Pre-capillary pulmonary hypertension complicating the CREST syndrome: A case report.

Pre-capillary pulmonary hypertension was the presenting sign of a CREST syn drome in a 65 year old woman. The diagnosis of this form of scleroderma is based on the association of a number of features (calcinosis, Raynaud's phe nomenon, oesophageal dyskinesia, sclerodactylia and telangectasia). Sclerod erma is the systemic disease which is usually complicated by pre-capillary pulmonary hypertension. This complication is observed in about 13% of CREST syndromes, but very rarely as severe pre-capillary pulmonary hypertension. The diagnosis of pre-capillary pulmonary hypertension carries a poor progn osis with a 2 year survival rate of about 40%. Treatment is usually with calcium inhibitors but with no effect on prognosi s. The use of prostacycline and its analogue, iloprost, is an interesting t herapeutic strategy, currently under evaluation. Cardiopulmonary transplant ation is the only treatment of very severe forms, despite the progressive c haracter of the condition. All cases of pre-capillary pulmonary hypertensio n require complete aetiological investigation to exclude a systemic disease , especially a scleroderma and, above all, a CREST syndrome.