Mitomycin Induced Pulmonary Veno-Occlusive Disease

Document Type

Conference Proceeding

Publication Date

5-17-2022

Publication Title

Am J Respir Crit Care Med

Abstract

Introduction: Pulmonary veno-occlusive disease [PVOD] is a very rare cause of group 1 pulmonary hypertension (PH) with no proven medical therapy. The only confirmatory test is a lung biopsy. Having a high suspicion when there is evidence of septal thickening, lymphadenopathy, and centrilobular ground glass nodules on computed tomography (CT) as well as a severe reduction in diffusion capacity on pulmonary function testing (PFT) is crucial. Here we describe a case of PVOD secondary to mitomycin therapy. Case Presentation: A 59-year-old female with a history of stroke and active anal squamous cell carcinoma treated with radiation and mitomycin who presented for pulmonary follow up. She was recently hospitalized for hypoxia attributed to mitomycin induced non cardiogenic pulmonary edema. An echocardiogram found a severely enlarged right ventricle with reduced function, an elevated PA systolic pressure of 58, and CT chest demonstrated a nonocclusive segmental pulmonary embolism. A right heart catheterization revealed a mean wedge pressure of 4, mean pulmonary artery pressure 37, and elevated pulmonary vascular resistance. An autoimmune workup and ventilation perfusion scan were unremarkable. PFTs demonstrated normal lung volumes, no obstruction in airflow, and a severe reduction in diffusing capacity. High resolution CT chest demonstrated septal thickening, centrilobular ground glass nodules, scattered areas of peribronchial consolidation, and mosaicism with minimal air trapping. Her PH was deemed to be likely due to PVOD with associated small airway disease from mitomycin therapy, for which she was not a candidate for lung transplant due to her active cancer. Case Discussion: Mitomycin has been associated with acute lung injury, interstitial pneumonitis, PVOD, and inducing bronchospasm. The importance of recognizing PVOD is underscored by the high one-year mortality, lack of proven medical therapy, and the need for definitive treatment with transplantation. Treatment with pulmonary vasodilators can lead to life threatening pulmonary edema in 50-75% of cases. A high index of suspicion must be maintained to avoid adverse outcomes and delay of definitive treatment. The clinical presentation, findings on CT imaging of the chest, and severe reduction of diffusion capacity make the likely unifying diagnosis for her presentation PVOD caused by mitomycin therapy.

Volume

205

Issue

1

First Page

A4457

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