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Background: Behçet’s disease (BD) is a multisystem vasculitis and pulmonary involvement in BD is reported to indicate poor prognosis and high mortality. Objectives: The aims of this study were to report on patients with pulmonary involvement and to discuss pulmonary artery thrombus and small-sized vasculitis associated with BD, with respect to previously published cases. Methods: Fifteen patients with BD and pulmonary involvement were included in this study. Massive hemoptysis was observed in all patients having pulmonary artery aneurysm (PAA). Results: Eleven patients had macroscopic pulmonary vascular disease (2 PAA, 3 PAA and thrombi and 6 only thrombi) and 3 patients had microscopic pulmonary vascular disease. The remaining patient had pulmonary cryptococcosis. Conclusions: Data regarding treatment and outcomes of patients having BD-related pulmonary emboli/infarct and small-sized vasculitis are limited. Pulmonary vasculitis affects different levels of the pulmonary artery in BD and should be classified as macroscopic and microscopic vascular disease. ‘Pulmonary artery thrombosis’ should be used instead of ‘pulmonary emboli’. Spiral CT angiography is the best radiological tool for evaluation of pulmonary problems in BD. Treatment of vasculitis should be based on the type of vascular disease and may vary among different types of vascular disease. Anticoagulation can be used in patients with microscopic vascular disease and nonaneurysmal macroscopic vascular disease. More studies are needed to clarify this issue.

Lung cancer, pulmonary embolism and bronchiectasis were the main causes of hemoptysis in this prospective cohort; however, this is the first report showing pulmonary embolism as a leading cause of hemoptysis. CT angiography with high-resolution CT should be the primary diagnostic modality if the initial investigation is inconclusive in hemoptysis cases.

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