Pulmonary involvement in patients with Behçet's disease: report of 15 cases

Behçet's disease (BD) is a multisystem vasculitis, and pulmonary involvement in BD can have complex clinical manifestations. We aimed to analyze the clinical and radiological features, as well as the outcomes, of BD patients with pulmonary manifestation.

We retrospectively reviewed the medical records of 106 patients with BD diagnosed in our institute between January 2000 and January 2013. Patients with pulmonary vascular or parenchymal abnormalities on chest radiography, thorax computed tomography, magnetic resonance imaging or pulmonary scintigraphy were included in this study.

Fifteen patients (14%) were identified to have pulmonary involvement. Pulmonary artery aneurysms (PAA) was observed in six patients, and all of them had concomitant thrombi, attenuation or occlusion of PAs, and five of them also had radiographic parenchymal changes. Three patients had solely pulmonary artery thrombus (PAT) without PAA. For the six patients with isolated radiographic parenchyma changes, pulmonary infiltration resolved with immunosuppressant therapy in four subjects; the lesion remained unchanged in one subject with radiographic interstitial changes, and acid-fast bacilli were found in the remaining subject. Patients with PAA or PAT had more frequency of hemoptysis and extra-pulmonary vascular lesions compared with isolated parenchymal involvement. Radiographic parenchyma changes are nonspecific, with ill-defined ground-glass opacity being the most common pulmonary radiographic parenchymal changes. Patients with isolated parenchymal changes had better prognosis than those with PAA or PAT.

BD with pulmonary involvement can have a wide spectrum of abnormal clinical and radiographic manifestations, and multiple pulmonary lesions can exit in the same patient.