A Rare Case of Pulmonary Veno-Occlusive Disease, Pulmonary Capillary Hemangiomatosis

Abstract

A 40-year-old male with past medical history of chronic pain on methadone for a few years, hypertension, and obesity presented with worsening dyspnea and was found to have significant hypoxemia. A CT-angiogram of the chest showed extensive diffuse bilateral pulmonary infiltrates with bilateral hilar and mediastinal lymphadenopathy. Extensive work-up exploring infectious and rheumatologic etiologies was unremarkable. Patient was empirically treated with IV antibiotics, high dose steroids and aggressive diuresis without significant clinical improvement. Further more, serial chest radiographs showed no improvement. Right side heart catheterization revealed normal pulmonary wedge pressure with elevated right-side pressure consistent with pre-capillary pulmonary hypertension. Right video assisted thoracoscopic surgery (VATS) wedge lung biopsy was done and tissue biopsy revealed marked intra-alveolar congestion by red blood cells, patchy aggregates of pulmonary macrophages within these alveolar spaces, and thickened alveolar septa. The thickened alveolar septa contained an increased number of dilated capillaries and mild numbers of chronic inflammatory cells. Alveolar macrophages contain anthracotic pigment as well as occasional pigment compatible with hemosiderin. There was no evidence of significant fibrotic change, fibroblastic foci, granulomas, or other significant inflammatory changes. This is consistent with capillary hemangiomatosis like change consistent with chronic venous hypertension.