Chylothorax and Thrombosed Obtuse Marginal Artery as Initial Manifestations of Behcet's Disease

Abstract

INTRODUCTION: The diagnosis and management of an inflammatory multisystem vasculitic disorder can be challenging in a critically ill patient. CASE PRESENTATION: A 23-year-old male presented with fevers, night sweats, a left neck mass and face and upper extremity swelling. He had no prior history of oral or genital ulcers. CT-thorax revealed bilateral pleural effusions, mediastinal and cervical LAP, with right brachiocephalic and subclavian vein occlusion and a clot in the SVC. Bone marrow biopsy and peripheral smears were nonrevealing. Pleural fluid analysis suggested chylothoraces. Despite extensive collaterals, mediastinoscopy was attempted but abandoned due to extreme mediastinal fibrotic changes. Right VATS for mediastinal biopsies revealed significant fibrosis. Postoperatively steroids were used to ameliorate airway edema and facilitate extubation. Left supraclavicular LN and EBUS guided mediastinal LN biopsies were also negative for malignancy. Repeat CT revealed a new left ventricular free wall cardiac mass adjacent to the atrioventricular groove. Cardiac MRI showed a well-defined 4.5cm spherical mass in the AV groove consistent with a thrombosed coronary artery pseudo-aneurysm. Shortly after weaning steroids, he became febrile with new oral aphthous ulcers as well as anterior chest wall ulcers. Broad spectrum antibiotics were initiated. Pleural fluid, oral ulcer swab and blood cultures were negative. Oral ulcer biopsy revealed superficial and perivascular inflammation. His clinical presentation was consistent with Behcet's disease(BD), with the presentation of ulcers, which were masked initially by the steroids. He was started on cyclophosphamide with steroids. He subsequently underwent ligation of the thoracic duct as well as thoracic duct and tributary platinum coil embolization. Currently, his effusions remain small, he no longer has signs of SVC syndrome, and the cardiac mass has reduced in size as he continues to be on immunosuppressive agents. DISCUSSION: Behcet's disease is a systemic inflammatory vasculitis of unknown etiology, characterized by relapsing episodes of orogenital ulcers, skin lesions, ocular lesions and rarely vascular, gastrointestinal and neurological involvement. Pulmonary involvement is uncommon (1-7.7% cases) and presents as thromboangiitis, pulmonary artery aneurysms, recurrent pneumonias and BOOP. Chylothorax as an initial manifestation of BD is extremely rare and is hypothesized to be secondary to large vein occlusion, such as SVC thrombosis causing lymphatic obstruction. The spectrum of cardiac diseases may include pericarditis, coronary artery stenosis or aneurysm, myocarditis, cardiomyopathy, valvular pathology and aneurysm of aorta and its branches. There are no laboratory findings specific for BD. CONCLUSIONS: The diverse clinical manifestations of BD can be challenging especially with an atypical presentation.