PULMONARY CAPILLARY HEMANGIOMATOSIS AS A RARE CAUSE OF PULMONARY-HYPERTENSION

Pulmonary capillary hemangiomatosis (PCH), first described by Wagenvoo rt et al. in 1978(11), is a rare cause of pulmonary hypertension which occurs predominantly in young adults. In the literature only 18 cases have been reported(3,4,6,7,9-11,14). In 1988 Langleben et al.(7) prop osed a hereditary form of PCH with probable autosomal-recessive transm ission. Histologic findings include irregular small nodular foci of th in-walled capillary-sized vessels which diffusely invade the lung pare nchyma, the bronchial/bronchiolar walls and the adventitia of large ve ssels. We report on a case of PCH in a 24 year old man who presented t he clinical signs of interstitial lung disease.