Multiple pulmonary arterial aneurysms in Behçet's disease: clinical and radiologic remission after cyclophosphamide and corticosteroid therapy.

ABSTRACT

Behçet's disease (BD) with pulmonary arterial aneurysm is rare and often associated with a poor prognosis. But there is also a chance that the aneurysm may completely resolve with medical therapy. A 39-year-old man presented with fever, malaise, bilateral chest pain, recurrent oral ulcers, and hemoptysis. The chest radiograph showed a round opacity in the right hilum. Computed tomography and magnetic resonance angiography (MRA) further revealed multiple, bilateral pulmonary arterial aneurysms consistent with the diagnosis of BD. The patient was started on a course of cyclophosphamide and corticosteroid therapy that resulted in cessation of his symptoms and complete resolution of radiologic findings. The chest radiograph and MRA reverted to normal on long-term follow-up. He is still alive and symptom-free 20 months after diagnosis.