Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension, regarded by some as distinct entities. However, their presentations are similar and both are associated with poor prognoses. We therefore reviewed 38 specimens autopsies (n=15), surgical biopsies (n=15), explants (n=7), and pneumonectomy (1 case) from 35 patients diagnosed as either PVOD (n=30; av. age 34 y, range 4 to 68 y; 19M:11F) or PCH (n=5, av. age 42 y, ranging from 9 months to 60 years; 3M:2F) to assess their interrelationship. PCH was identified in 24 (73%) cases diagnosed as PVOD, either as perivenular foci or diffuse involvement of the pulmonary parenchyma. Other features seen in PVOD were arterial medial hypertrophy and/or intimal fibrosis (88%), hemosiderosis (79%), venulitis (12%), infarction (9%), interstitial fibrosis (sometimes as localized scars) (48%), and a mild lymphocytic infiltrate (67%). In cases diagnosed as PCH, 4 showed venous and arterial changes of PVOD. Cases with PCH also all showed a mild interstitial lymphocytic infiltrate but there was no venulitis or infarction. Capillary proliferation was particularly well demonstrated by CD34 immunostaining and predominantly involved the alveoli, but was also seen within walls of bronchi and pulmonary vessels. Our data suggest that in the majority of cases PCH represents a secondary angioproliferative process caused by postcapillary obstruction rather than a separate disease. The cause of the venous obliteration was not identified but the occasional identification of phlebitis suggests this plays a role in venous damage in some cases.