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A young Turkish woman with dry cough

Behçet’s disease (BD) is a multisystem disorder characterized by vasculitis. The disease consists of a triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. Since its first description, involvement of other organs has been described. Skin, central nervous system, and gastrointestinal and pulmonary involvement are among those reported. A 33-year-old woman with BD presented with dry cough for 20 days. Chest radiograph (Fig. 1) revealed bilateral rounded opacities on both hilar regions, more prominent on the left, which was confirmed to be a large pulmonary artery aneurysm (PAA) of the left and smaller one on the right on spiral computed tomographic angiography (Fig. 1). She was treated with 60 mg of prednisolone daily and 1 g pulse cyclophosphamide once a month for 6 months. Complete clinical and radiological response was achieved with this treatment (Fig. 2). No recurrence was observed 25 months after diagnosis and she has been maintained on 5 mg of prednisolone and 100 mg of azathioprine. Involvement of the pulmonary arteries is rare in other systemic vasculitides, but PAA is the most feared and most common arterial complication of BD with a mean survival of 10 months. It usually affects young male patients. Massive haemoptysis is the most common symptom and the usual cause of death in BD with pulmonary involvement. Early diagnosis and prompt immunosuppresive treatment may prevent a fatal outcome.