title: Pulmonary capillary hemangiomatosis associated with connective tissue disease: a report of 4 cases and review of the literature.
abstract: Pulmonary hypertension (PHTN) can be seen in patients with connective tissue disease (CTD). The typical pathology associated with CTD is interstitial fibrosis and hypertensive pulmonary arteriopathy. We describe 4 patients with CTD and PHTN unexpectedly found to have pulmonary capillary hemangiomatosis (PCH) at explant after lung transplantation or autopsy. Pulmonary capillary hemangiomatosis is defined as a proliferation of capillaries in alveolar walls and can clinically cause PHTN. We detail the pathologic findings of PCH, describe the differential diagnosis, and present a review of the literature on the possible association of PCH with CTD. Although PCH may present clinically as PHTN, it is critical to differentiate between the typical CTD-associated interstitial fibrosis with hypertensive pulmonary arteriopathy and PCH because the treatment is different. We provide the largest case series to date and highlight the need for pathologists to have a high level of suspicion for PCH in patients with CTD.
authors.affiliation: Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH.
authors.forename: Shelley I
authors.initials: SI
authors.lastname: Odronic
authors.affiliation: Pulmonary, Allergy, and Critical Care, Cleveland Clinic, Cleveland, OH.
authors.forename: Tathagat
authors.initials: T
authors.lastname: Narula
authors.affiliation: Pulmonary, Allergy, and Critical Care, Cleveland Clinic, Cleveland, OH.
authors.forename: Marie
authors.initials: M
authors.lastname: Budev
authors.affiliation: Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH. Electronic address: farverc@ccf.org.
authors.forename: Carol
authors.initials: C
authors.lastname: Farver
mesh_terms: D000328:Adult; D002196:Capillaries / Q000473:pathology; D003240:Connective Tissue Diseases / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D018324:Hemangioma, Capillary / Q000473:pathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000473:pathology; D008168:Lung / Q000473:pathology; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000188:drug therapy / Q000473:pathology
keywords: connective tissue disease; pulmonary capillary hemangiomatosis; pulmonary hypertension; scleroderma