Rare rheumatic disorders. A. Behçet's disease

No more than 100 patients with respiratory involvement in Behçet's disease have been described in the literature. Haemoptysis, cough, dyspnoea and pleuritic chest pain are the main symptoms. Vasculitis and thrombosis of the great pulmonary vessels is the underlying condition in the majority of patients. Aneurysmal formation and rupture is possible and constitutes the most life-threatening aspect of the disease. Behçet's syndrome should be considered in the differential diagnosis of haemoptysis and veno-occlusive disease, especially where large vessels are involved. Early detection and treatment is mandatory to save the patient's life. Other pleuropulmonary manifestations described, such as fibrosing alveolitis and pleural disease, occur less frequently in Behçet's disease, and carry a better prognosis.