Pulmonary disease in Behçet's syndrome

Abstract

Five patients with Behçet's syndrome presenting with haemoptysis and recurrent radiographic opacities are reported, with a review of 23 similar cases. All 28 cases conformed to a definite clinical pattern with haemoptysis, usually accompanied by pyrexia, chest pain and dyspnoea, being the major feature. Typically pulmonary disease was associated with active disease at other sites, although the patients often only complained of haemoptysis. Patients with Behçet's syndrome and haemoptysis, compared to those without haemoptysis, showed a marked male predominance, with thrombophlebitis and deep vein thrombosis being more common. Rarely pulmonary disease occurred in the absence of one or other of the so called 'major' criteria on which the diagnosis of Behçet's syndrome is usually made, as was so for four of our patients who did not have ocular disease. Immunopathological evidence suggests that the underlying pathogenesis is a pulmonary vasculitis which may result in arterial and venous thromboses, pulmonary infarction, pulmonary haemorrhage and pulmonary arterial aneurysm formation. A role for immune complexes in the pathogenesis of pulmonary disease in Behçet's syndrome is suggested by the finding of circulating immune complexes in association with active pulmonary disease. Corticosteroids were initially helpful in treating active disease in the lungs, and at other sites, in most of the patients in whom they were tried, including our five patients, but serious haemoptysis occasionally recurred, despite further treatment. Four of our patients were initially treated with anticoagulants for a presumed diagnosis of pulmonary embolism, but continued to have haemoptysis. One of these patients subsequently died following massive haemoptysis, despite good anticoagulant control. The haemoptysis in most of the 28 cases was notable for its severity, and in at least 11 (39.3 per cent), pulmonary haemorrhage was the probable cause of death. All deaths due to haemoptysis occurred within six years of the first episode. Whilst pulmonary disease with haemoptysis is infrequent, it carries a very serious prognosis and for this reason correct diagnosis and appropriate treatment is imperative. Pulmonary disease with haemoptysis should be included as one of the so called 'minor' criteria in the diagnosis of Behçet's syndrome.