CTICasesGranulomatous Venulitis
Imaging findings
Chest CT in a 17-year-old male with progressive dyspnea and severe hypoxemia shows diffuse peribronchovascular thickening, centrilobular nodules, ground-glass opacities, and small pleural effusions. Right heart cath shows pulmonary hypertension. Surgical lung biopsy demonstrates a granulomatous venulitis (granulomatous angiitis) characteristically obliterating the intermediate-sized pulmonary veins, with secondary features of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH).
Key takeaways
Granulomatous venulitis (or granulomatous angiitis of the pulmonary veins) is an extremely rare, idiopathic vascular disease that can present as a form of pulmonary veno-occlusive disease (PVOD). The granulomatous inflammation selectively target and obliterate the small-to-intermediate pulmonary veins, leading to post-capillary pulmonary hypertension. Differentiating this from other vasculitides is difficult and requires surgical biopsy.
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