Bilateral pleural effusions in a 23-year-old man: a clinical puzzle.

A 23-year-old man presented with progressive swelling in the left supraclavicular area and worsening dyspnea of 7 days duration. Histories of fever, chills, night sweats, cough or weight loss was denied. Medical and family histories were unremarkable. Physical examination revealed usage of accessory muscles of respiration, facial plethora, acne, multiple dilated veins on chest wall, firm non-tender bilateral supraclavicular lymph nodes and decreased air entry and dullness in both lung bases. Chest X-ray revealed bilateral pleural effusions (Figure 1A). Computed tomography (CT) scan of the neck and chest demonstrated thrombotic occlusion of the superior vena cava, right subclavian, innominate and internal jugular veins, mediastinal fibrosis, subcentimeter mediastinal adenopathy and a mass on the left cardiac atrioventricular wall (Figure 1B and C). Pleural fluid revealed a triglyceride concentration of 799mg/dl and lactate dehydrogenase (LDH) of 947U/l with negative cultures and cytology. Lymph node and pleural biopsies were benign. Cardiac magnetic resonance imaging revealed a cystic 3.1 3.1 cm mass, which was delineated to be a thrombosed psuedoaneursym of the left circumflex coronary artery by cardiac catheterization. During hospitalization, the patient developed oral aphthous ulcers and folliculo-pustular rash on chest. Biopsy revealed superficial and perivascular inflammation. A positive pathergy test followed, clinching the elusive diagnosis of Behcet’s disease. The patient was started on anticoagulation along with cyclophosphamide and prednisone. For the persistent chylothorax, triglyceride restriction, chest tubes with attempts at pleurodesis proved futile. After an attempt at thoracic duct embolization, ligation and partial decortication of the right lung were performed. Six months into follow-up, the patient still has a loculated right-sided pleural effusion, with the disease in remission.