We report a case of Behcet's disease with pulmonary and splenic involvement.

We report a case of Behcet's disease with pulmonary and splenic involvement. The radiologic presentation was quite different from the fashions defined in the literature. A large, anteriorly located, well circumscribed ovoid mass was the x-ray film image of a large area of infarction with hemorrhage. The involved pulmonary artery showed signs of vasculitis with thrombus formation. To our knowledge, this manner of presentation is different from the cases reported in the literature.

Behcet's disease has worldwide distribution, nevertheless Orientals and those from the Eastern Mediterranean countries have the highest incidence of the disease.[1] Pulmonary manifestations of the disease are well described in several series.[2] In this report, we describe a patient with a mass lesion in the right lung who concealed the diagnosis of Behcet's disease 2 years earlier, based upon neurologic symptoms.

With this case in mind, we glance at a review of Behcet's disease in patients with a mass lesion of the lung especially in the geographic areas mentioned previously

Case Report

A 41-year-old man was admitted to the pneumology clinic with renewed episodes of hemoptysis, dyspnea, and chest pain. He gave a history of splenectomy done for splenic abscess 6 month ago. Physical examination was normal save for diminished breath sounds above the right chest wall. The erythrocyte sedimentation rate was 80 mm/h A chest x-ray film revealed a large, well-circumscribed ovoid mass lesion of the right upper and middle lung fields. springs of sputum culture and cytology were negative for malignancy. The comput tomography scan of the chest revealed a 53 x 61 mm mass lesion in the right upper lobe. Nothing pathologic was observ during bronchoscopy leave out extrinsic compression of the intermediate carina.

ends of a biopsy specimen taken from this area and lavage fluid were inconclusive. Because the mass had a 166 HU density, transthoracic needle aspiration biopsy was performed with comput tomographic guidance. Biopsy material showed inflammatory small rooms The patient was transferred to the department of thoracic and cardiovascular surgery for exploratory thoracotomy. The night before the operation, he experienced hemoptysis (1000 ml) which ceased spontaneously.

The operation was carried in succession as scheduled on the following day. A well encapsulated, stretched 10 x 10 cm mass was seen occupying the anterior portion of the right upper lobe. There was no pulsation. We decided to do an upper lobectomy and refrained from taking a sample for frozen section because the consistency of the mass gave us the impression that it could easily split open and contaminate the field. The capsula was torn during the dissection, liberating elderly and new thrombus with bleeding. An upper lobectomy was complet subject to normal conditions. His postoperative course was uninteresting and he was discharged forward the 10th day.

The pathologic examination revealed a large area of infarction. Pulmonary artery branches adjacent to this area showed massive mononuclear solitary abode; squalid infiltration with fresh thrombus formation. In single of the pulmonary arteries, local dilation with elastic fiber degeneration and organized thrombus was apparent. Surrounding lung tissue showed lobular pneunomonitis with intra-alveolar hemorrhage, edema, and condensed fibrosis (Fig 1).

The patient then was readmitted and a more detailed history was taken. He told that he had been receiving treatment with the diagnosis of neuro-Behcet's disease and had stopped taking his medications 2 years ago. His preoperative chest x-ray film before splenectomy demonstrated no pathologic abnormalities.

Physical examination revealed genital ulceration and scarring. With the

history of oral ulceration and positive reaction, diagnosis of Behcet's disease was made according to the International subject of attention Group criteria.[3] Meanwhile, his medical report obtained from the neurology department showed that he had bilateral uveitis, folliculitis, and oral and genital ulcerations. Moreover, bilateral sigmoid sinus thrombosis was demonstrated with angiographic and comput tomographic studies of the brain.

When reviewing his medical report referring to splenectomy, it was learned that he was admitted to that hospital with the clinical findings of heat and abdominal pain. A comput tomography scan of the abdomen demonstrated a splenic mass with liquid density 5 cm in diameter. Splenectomy was done with the diagnosis of splenic abscess, which was supported with pathologic study

Reexamination of the chagrin blocks after the diagnosis of Behcet's disease disclosed a large area of infarction with mononuclear enclosed space infiltration of the surrounding trabecular artery. Organized and recanalized thrombus was exposeed in this artery. Thickening of the trabecular artery and central arterial wall with hyalin deposits were noted (Fig 2)

Therapy with cyclophosphamide and corticosteroids was started. Three month later, DSA subject of attention of the pulmonary arteries demonstrated a small aneurysm of the right lower lobe artery. As a dependence of cause and effect of poor pulmonary function experiment values (vital capacity, 35 percent; [FEVsub1] 76 percent) a decision was made to continue medical therapy with cyclophosphamide and prednisolone. This sign of therapy was carried on the outside with success by Stricker et al.[4] Although the size of the aneurysm did not change during the follow-up period, this patient died of massive hemoptysis.

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