Chest
Roentgenogram of the MonthHilar Mass and Papilledema on Presentation
Section snippets
What is the diagnosis?
Diagnosis: Behçet's syndrome (BS) with pulmonary artery aneurysm (BAA) and venous thrombosis.
The clinical impression was BS with PAA. This was confirmed with CT scans (Fig 2) and MRI scans of the chest (Fig 3) which showed a 2-cm right PAA, a 1.5- to 2.0-cm peripheral aneurysm in the lower lobe of the left lung, and multiple ill defined nonspecific small peripheral lung opacities probably representing small areas of infarction. Magnetic resonance angiography of the brain (Fig 4) showed
Discussion
The clinical triad of uveitis and oral and genital ulcers was recognized by Hippocrates, but bears the name of Hulusi Behçet who described it nearly 60 years ago. The disease is systemic, with variable involvement of many organ systems. The cause is unknown and there is no universally accepted diagnostic test.
Etiology and Pathogenesis
BS is a multisystem vasculitis of unknown cause. It affects virtually all sizes of arteries and veins. It also may be accompanied by thromboembolism of the superior vena cava and mediastinal veins. The pulmonary vascular tree is involved with significant morbidity. BS, of all nonspecific vasculitides, is the most common cause of PAAs, which frequently rupture and cause devastating hemorrhage.1, 2 In a case series of 2,179 patients, 24 patients were found to have PAAs.3 There was a high
Diagnosis
BS has a strong male predominance. The International Study Group for Behçet's Disease proposed the following five criteria for the diagnosis:9
- 1.
Recurrent oral ulceration: minor aphthous, major aphthous, or herpetiform ulcers which have recurred 3 times in a 12-month period.
Plus any two of the following:
- 2.
Recurrent genital ulceration noticed by physician or the patient.
- 3.
Eye lesion: anterior or posterior uveitis, cells in vitreous on slitlamp examination, or retinal vasculitis observed by an
Prognosis and Treatment
In one large study,3 50% of patients died at a mean of 9.5 months after the onset of hemoptysis, implying that PAA carries a high short-term mortality despite treatment. The aneurysms may regress or even completely disappear with corticosteroid treatment.3, 5, 12 Various treatment modalities have been described with variable results. These include surgery, embolization of the aneurysm, immunosuppressive drugs such as cyclophosphamide, azathioprine, and cyclosporine alone or in combination with
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