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.
Keywords: connective tissue disease; pulmonary capillary hemangiomatosis; pulmonary hypertension; scleroderma.
Copyright © 2015 Elsevier Inc. All rights reserved.