DOI: 10.33706/jemcr.1849453 ISSN: 2149-9934

GIANT ANEURYSMS OF THE LAD AND CX ARTERIES ASSOCIATED WITH BEHCET’S DISEASE: A VERY RARE ASSOCIATION

Zeynep Demir, Yakup Alsancak, Muhammed Fatih Kaleli, Ahmet Seyfeddin Gürbüz, Ahmet Soylu
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Behçet’s disease is a chronic multisystem inflammatory disorder characterized by vasculitis affecting vessels of all sizes. Although vascular involvement is common, coronary artery aneurysms are rare and associated with significant morbidity and mortality, particularly in young patients. In the emergency department, young adults presenting with acute chest pain and myocardial injury without classical atherosclerotic risk factors represent a diagnostic challenge. This case emphasizes the importance of considering inflammatory vasculitic disorders in the differential diagnosis of acute coronary syndromes. Introduction Behçet’s disease (BD) is a chronic inflammatory condition with multisystem involvement, most commonly affecting the vascular system. Typical manifestations include recurrent oral and genital ulcers, ocular inflammation, and skin lesions. Vascular involvement is a major cause of morbidity and mortality. Cardiac involvement, though uncommon, may present as myocardial infarction secondary to coronary arteritis and has been reported in up to 17% of patients with cardiac involvement. In approximately 25% of cases, cardiac manifestations may be the initial presentation. Case Report A 23-year-old male presented to the emergency department with acute retrosternal chest pain radiating to the neck and shoulder, worsened by inspiration and positional changes. He had no significant cardiovascular risk factors except smoking. Electrocardiography was unremarkable. Transthoracic echocardiography showed anterior and lateral left ventricular hypokinesia with an ejection fraction of 45% and minimal pericardial effusion. Laboratory evaluation revealed elevated troponin and leukocyte levels. The patient was initially treated for presumed myopericarditis. Coronary angiography revealed a large aneurysm and pseudoaneurysm of the proximal left anterior descending artery with severe distal stenosis, along with a saccular aneurysm of the circumflex artery. Further evaluation demonstrated genital scars and a positive pathergy test, supporting the diagnosis of Behçet’s disease. Discussion Coronary artery aneurysm is a rare but serious manifestation of BD, caused by inflammatory destruction of the arterial wall. The prevalence of coronary involvement is approximately 0.5%. Patients are typically young males without traditional cardiovascular risk factors . For emergency physicians, BD should be considered in young patients presenting with chest pain, myocardial injury, or recurrent pericarditis when atherosclerotic disease is unlikely. Early recognition in the emergency department is crucial to prevent diagnostic delay and inappropriate management. Conclusion Behçet’s disease should be included in the differential diagnosis of acute chest pain in young patients presenting to the emergency department. Prompt diagnosis, immunosuppressive therapy, and multidisciplinary management are essential to reduce morbidity associated with coronary involvement.

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