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A3069 - Pulmonary Arteritis Mimicking Recurrent Pulmonary Embolism in a Patient with Bechet’s Disease
Author Block: T. Plush; Lankenau Medical Center, Philadelphia, PA, United States.
Introduction: Bechet’s disease is an inflammatory autoimmune disorder marked by recurrent oral aphthae and various other systemic manifestations. Most of these sequelae are thought to be due to vasculitis that occurs in blood vessels of all sizes. We present a case of pulmonary arteritis mimicking pulmonary thromboembolism in a patient with Bechet’s disease. Case Overview: 51-year-old male with a recent diagnosis of Bechet’s disease, who was lost to follow up and noncompliant presented with left neck swelling. He also had a previous pulmonary embolism (PE), Budd Chiari, carotid arteritis status post carotid stenting, and multiple cerebral infracts. He presented with a pulsatile mass in his neck and was diagnosed with a carotid artery aneurysm and underwent stenting. Post-operatively, he developed neurologic changes, and an MRI showed new acute infarcts. Repeat CT angiography of his chest due to pleuritic chest pain without any complaint of hemoptysis demonstrated filling defects in the same distribution as his previous PE. Intravenous heparin was started duepossible recurrent PE, without any significant bleeding. He was also treated with pulse methylprednisolone cyclophosphamide. At discharge, he was given prednisone and rivaroxaban. Discussion: Bechet’s disease is an autoimmune disease characterized by systemic vasculitis of blood vessels of all sizes. Only small case series have described the various pulmonary manifestations that can occur. The most common sequelae are PAA, often presenting with hemoptysis.1 The underlying pathophysiologic mechanism is related to vasculitis, which may result in thrombosis, infarction, hemorrhage, and PAA formation.1 Anticoagulation in the setting of PAA has been reported to cause worsening of bleeding and possible death, therefore, initiation of immunosuppression is recommended prior to anticoagulation. Thrombosis is common in Bechet’s disease, often at unusual sites such as cerebral sinuses or hepatic veins, however PE is rarely described.2 A small retrospective study found no difference in recurrent thrombosis in patients treated with anticoagulation compared with those treated with immunosuppressive therapy alone.3 Endothelial dysfunction and altered coagulation also play a role, however, thrombi within the pulmonary vasculature were almost always associated with pulmonary vasculitis or an aneurysm1,4 In conclusion, the approach to possible PE in a patient with Bechet’s disease must be approached with caution, as pulmonary arteritis is a much more likely diagnosis, and the initiation of anticoagulation prior to immunosuppression has been associated with bleeding and death.