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

Öz

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. 

Anahtar Kelimeler

• behcet's disease,
• coronary artery aneurysm,
• myocardial infarction

Etik Beyan

Written informed consent could not be obtained because the patient could not be reached. Therefore, this case report was prepared using fully anonymized data. No information that could identify the patient or reveal confidential details is included in the manuscript. Detailed clinical data are available to the editor and reviewers upon request.

Behçet Hastalığına Bağlı LAD ve CX arterde Dev Anevrizma; Çok Nadir Bir Bİrliktelik

Öz

Behçet hastalığı, tüm çaplarda damarları etkileyebilen vaskülit ile karakterize kronik, multisistemik inflamatuvar bir hastalıktır. Vasküler tutulum sık görülmesine rağmen, koroner arter anevrizmaları nadirdir ve özellikle genç hastalarda ciddi morbidite ve mortalite ile ilişkilidir. Acil servise aterosklerotik risk faktörleri olmaksızın akut göğüs ağrısı ve miyokard hasarı ile başvuran genç hastalar tanısal zorluk oluşturur. Bu olgu, akut koroner sendrom ayırıcı tanısında inflamatuvar vaskülitik hastalıkların önemini vurgulamaktadır. Giriş Behçet hastalığı (BH), en sık vasküler sistemi tutan kronik inflamatuvar bir hastalıktır. Tekrarlayan oral ve genital ülserler, oküler inflamasyon ve cilt lezyonları tipik bulgulardır. Vasküler tutulum, hastalığın morbidite ve mortalitesinin başlıca nedenidir. Kardiyak tutulum nadir olmakla birlikte, koroner arterit sonucu miyokard enfarktüsü şeklinde ortaya çıkabilir ve kardiyak tutulumlu hastaların %17’sine kadarında bildirilmiştir. Bazı olgularda kardiyak bulgular hastalığın ilk belirtisi olabilir. Olgu Sunumu Yirmi üç yaşında erkek hasta, acil servise inspirasyon ve pozisyonla artan, boyun ve omuza yayılan akut retrosternal göğüs ağrısı ile başvurdu. Sigara kullanımı dışında belirgin kardiyovasküler risk faktörü yoktu. Elektrokardiyografi normaldi. Transtorasik ekokardiyografide sol ventrikül anterior ve lateral duvarlarında hipokinezi, ejeksiyon fraksiyonu %45 ve minimal perikardiyal efüzyon saptandı. Troponin ve lökosit düzeyleri yüksekti. Hasta başlangıçta miyoperikardit ön tanısı ile tedavi edildi. Koroner anjiyografide sol ön inen arter proksimalinde büyük anevrizma ve psödoanevrizma ile ciddi distal darlık, ayrıca sirkumfleks arterde sakküler anevrizma izlendi. Genital skarlar ve pozitif paterji testi Behçet hastalığı tanısını destekledi. Tartışma Koroner arter anevrizması, BH’nin nadir ancak ciddi bir komplikasyonudur ve arter duvarındaki inflamatuvar yıkım sonucu gelişir. Koroner tutulum prevalansı yaklaşık %0,5’tir. Hastalar genellikle geleneksel kardiyovasküler risk faktörleri olmayan genç erkeklerdir. Acil serviste, özellikle genç hastalarda ateroskleroz dışı nedenler düşünülmeli ve Behçet hastalığı ayırıcı tanıda yer almalıdır. Sonuç Acil servise akut göğüs ağrısı ve miyokard hasarı ile başvuran genç hastalarda Behçet hastalığı akılda tutulmalıdır. Erken tanı, immünosupresif tedavi ve multidisipliner yaklaşım morbiditeyi azaltmada kritik öneme sahiptir. 

Anahtar Kelimeler

• BEHÇET HASTALIĞI
• KORONER ARTER ANEVRİZMASI
• MİYOKARD ENFARKTÜSÜ

Kaynakça

1. 1. Smith EL, Yazici Y: Clinical manifestations and diagnosis of Behçet’s syndrome. Merkel PA, Seo P (ed): UpToDate, Waltham, MA; 2023.
2. 2. Bettiol A, Alibaz-Oner F, Direskeneli H, Hatemi G, Saadoun D, Seyahi E, Prisco D, Emmi G. Vascular Behçet syndrome: from pathogenesis to treatment. Nat Rev Rheumatol. 2023;19:111–26.
3. 3. Manghera PS, Pradhan A, Bajpai J, Sethi R. Acute Myocardial Infarction as the Initial Presentation of Behcet’s Syndrome. Int J Appl Basic Med Res. 2020;10(4):292–4.
4. 4. Kariyanna PT, Shah P, Jayarangaiah A, Chowdhury YS, Lazaro D. Acute coronary syndrome in Behcet’s syndrome: A systematic review. Eur J Rheumatol. 2021;8(1):31–5.
5. 5. Atzeni F., Sarzi-Puttini P., Doria A., Boiardi L., Pipitone N., Salvarani C. Behçet’s disease and cardiovascular involvement. Lupus. 2005;14:9: 723-26. https://doi.org/10.1191/0961203305lu2208oa.
6. 6. Zhou J, Shi J, Liu J, et al. The clinical features, risk factors, and outcome of aneurysmal lesions in Behcet’s disease. J Immunol Res. 2019;2019:9198506
7. 7. Tekin B, Ozen G, Tekayev N, Gercek S, Direskeneli H. Acute coronary syndrome in Behcet’s disease caused by a coronary artery aneurysm and thrombosis. Eur J Rheumatol. 2014;1(4):156.
8. 8. Davatchi F. In: Textbook of Clinical Rheumatology. Hows HS, Feng PH, editors. National Arthritis Foundation; Singapore: 1998. Behcet’s disease; pp. 298–315. [Google Scholar]

9. 9. Shahram F, Davatchi F, Akbarian M, In: Behcet’s disease. Hamza H, editor. Pub Adhoua; Tunisia: 1997. The 1996 survey of Behcet’s disease in Iran, study of 3153 cases; pp. 165169. [Google Scholar]
10. 10. International Team for the Revision of the International Criteria for Behcet’s Disease (ITR-ICBD) The International Criteria for Behcet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28(3):338–347. doi: 10.1111/jdv.12107. [DOI] [PubMed] [Google Scholar]
11. 11. Matsumoto T, Uekusa T, Fukuda Y. Vasculo-Behcet’s disease: a pathologic study of eight cases. Hum Pathol 1991;22(1):45-51.
12. 12. Harrison A, Abolhoda A, Ahsan C: Cardiovascular complications in Behc¸et syndrome: acute myocardial infarction with late stent thrombosis and coronary, ventricular, and femoral pseudoaneurysms. Tex Heart Inst J 2009;36:498-500
13. 13. Hamzaoui A1, Bel Feki N, Brahem Sfaxi A, Smiti Khanfir M, Ben Ghorbel I, Lamloum M, Houman MH.Clin Exp Rheumatol. Chest pain in patients with undiagnosed Behçet’s disease.2012 May-Jun;30(3 Suppl 72):S76-9. Epub 2012 Oct 1.
14. 14. Kasapis C, Grossman PM, Chetcuti SJ: Cardiovascular flashlight. Percutaneous treatment of a giant right coronary artery pseudoaneurysm in Adamantiades-Behcet’s syndrome. Eur Heart J 2009;30:2630.
15. 15. Porcu P, Chavanon O, Bertrand B, et al: Giant aneurysm of the proximal segment of the left anterior descending artery in a patient with Behc¸et’s disease—a combined approach. Can J Cardiol 2008;24:e73-e74.
16. 16. Siegrist PT, Sumitsuji S, Osuga K, Sakaguchi T, Tachibana K, Nanto S.Endovascular coil embolization of Behçet disease related giant aneurysm of the right coronary artery after failure of surgical suture.JACC Cardiovasc Interv. 2013 Jun;6(6):e31-2.
17. 17. Gürkan U, Kaya A, Tatlısu MA, Avşar S.A case report of coronary artery aneurysm in a patient with Behçet’s disease. Turk Kardiyol Dern Ars. 2014 Oct;42(7):651-4.