Pulmonary vasculitis in Beh?et's disease.

Clinical findings of 12 patients with Beh?et's disease and lung involvement are presented. Male to female ratio was 11/1, mean age was 35.3 +/- 8.8 yr. All patients had at least four other organ manifestations of vasculitis, either in their history or during the period of lung involvement. The main complaint was hemoptysis of varying degree in 11 of the 12 patients. The chest X-ray films showed unilateral hilar enlargements in six patients, diaphragm elevation in four, horizontally or obliquely oriented linear opacities in three, diffuse, ill-defined infiltrates in upper and lower zones in three, wedge-shaped peripheral opacities in one, and bilateral pleural effusion in one patient. Computed tomography of the chest performed in nine patients revealed aneurysms, narrowings, and cutoffs of the main, lobar, segmental, or peripheral branches of the pulmonary artery and irregular configuration of other pulmonary vessels. Perfusion scans demonstrated defects of various sizes in all patients. Pulmonary angiography performed in only two patients showed amputation of branches of the pulmonary artery and aneurysmal dilatations. The patients were treated with a combination therapy consisting of corticosteroid, cyclophosphamide, colchicine, and antiaggregants, with very good results in the short term.