外科ジャーナル

Complete Resolution of Bilateral Pulmonary Artery Embolism and Aneurysmal Dilatation with Behcets Disease after Intravenous Cyclophosphamide Therapy Only: A Case Report and Review of the Literature

Abstract

Ahmed A. Abd Alkader, Ahmed K. Awad, Sheref A. Elseidy

Bechet syndrome is a multisystemic illness that leads to vasculitis. The classic constellation of symptoms is recurrent aphthous ulcers of the mouth and genitalia, hyperreactivity of the skin to penetrating trauma (pathergy reaction), other skin lesions, arthritis, and panuveitis. Vascular involvement can involve arteries and veins of all sizes and types. however venous involvement tends to be more common than arterial vasculitis mostly in the form of lower extremity vein thrombosis. Interestingly the disease manifestation of vasculitis itself seems to favor males than females and with a unique geographical distribution. Arterial vasculitis tends to affect the pulmonary vasculature, though Pulmonary involvement in Bechet’s disease is rare, occurring 1–7.7 of patients. Pulmonary artery aneurysms with or without “in situ" thrombosis is the most common form of arterial involvement and are the leading causes of increased mortality.

Different management modalities have been proposed. of these immunosuppressive therapies have shown to decrease the inflammation and lead to resolution of the aneurysmal dilatation itself. however, this treatment is reserved for patients with minor to moderate hemoptysis and who are vitally stable.

In this case, we report a patient with Behcet induced pulmonary artery aneurysm and in situ thrombosis who presented with massive hemoptysis and was managed with only immunosuppressive therapy after initial stabilization that leads to complete resolution of the aneurysm.