Pulmonary capillary hemangiomatosis (PCH) is a rare idiopathic lung disorder that occurs in young patients and leads to pulmonary hypertension (PH).
PCH is characterized by proliferating capillaries that invade the pulmonary interstitium and alveolar septae, and occlude the pulmonary vasculature.
Synopsis
capillary proliferations in the pulmonary alveolar walls
numerous cytologically benign thin-walled capillary-sized blood vessels proliferating diffusely through alveolar walls and in and around larger vessels and airways
venous infiltration with intimal fibrosis and secondary veno-occlusive disease
proliferation of benign thin-walled capillary sized blood vessels in the lung parenchyma
atypical endotheliomatosis
Associations
hereditary hemorrhagic telangiectasia (HTT) (14991548)
Variants
congenital pulmonary capillary hemangiomatosis (12910588)
diffuse bilateral pulmonary capillary hemangiomatosis after bilateral lung transplantation (12821168)
References
Tron V, Magee F, Wright JL, Colby T, Churg A. Pulmonary capillary hemangiomatosis. Hum Pathol. 1986 Nov;17(11):1144-50. PMID: 3770733