Abstract
In 1937, Hulusi Behcet’s brought to our attention the existence of a disease characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis [1-3]. He became the first physician to describe this and the disease was henceforth named after his name. The aetiology of this disease remains unclear however it has been noticed that Behcet’s syndrome is remarkable for its systemic vasculitides which include its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation with occlusive nature [4]. This makes Vasculitis the major cause of the clinical manifestations of the disease with the involvement of almost any organ. We herein report an unusual case of a thirty-year-old Syrian man with no past medical illnesses. On presentation, he suffered from a sudden onset of shortness of breath and a history of hoarseness of voice for less than a week, and drastic weight loss of about thirty kgs within a month. Imaging confirmed the presence of pulmonary embolism, bilateral deep venous thrombosis, intracardiac thrombosis, popliteal artery thrombosis with thoracic, and abdominal aortic aneurysms. This case highlights the importance of having a high suspicion of the possibility of Behcet’s disease when multiple thrombi and aneurysms manifest in a patient on the silk root country. Thereby, stressing the importance of familiarizing ourselves with such unusual presentations of Behcet’s disease and the course of this unusual presentation with some learning points with a good literature review included below.
Highlights
Behcet's disease is a multisystem inflammatory disorder with a chronic course
Intracardiac thrombus occurring as a thrombotic manifestation of Behcet's disease is an unusual presentation and represents a challenge in diagnosis and treatment [7]
The highest prevalence is in Asia, the Middle East, and Mediterranean areas which correspond to the high prevalence of the HLA-B51 allele
Summary
Isolated Aortitis with multiple Aortic aneurysms and widespread venous, arterial and intracardiac thrombosis: A challenging Vascular Behcet's Disease case management.
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