Two Vascular Multisystem Diseases with Pulmonary Involvement

Abstract

Also known as the Silk Road disease, Behçet disease (BD) has a worldwide distribution but is seen most frequently in Turkey, Japan, China, and the Mediterranean littoral. Men and women are affected equally. The disease is a triad of oral ulcers (Figure 28.1a, b), genital ulcers, and uveitis (Figure 28.2a, b, c). In a study consisting of 2179 patients, pulmonary involvement occurred in about 1.1%. Pulmonary vascular lesions lead to the formation of pulmonary artery aneurysms, the most common pulmonary lesion in BD almost always associated with hemoptysis, thrombosis and occlusion of the vessels, pulmonary infarction, and hemorrhage (Figure 28.3). Symptoms include dyspnea, cough, hemoptysis, and pleural effusion. Pathological changes include arterial aneurysm, arterial and venous thrombosis, infarction, and vasculitis (Figures 28.4 and 28.5).