DOI QR코드

DOI QR Code

Cardiac Behçet's Disease Presenting with Right Ventricular Endomyocardial Fibrosis and Intracardiac Thrombosis: a Case Report

  • Choi, Eun Ji (Department of Radiology and Research Institute of Radiology, Asan Medical Center) ;
  • Kim, Min Sun (Department of Radiology and Research Institute of Radiology, Asan Medical Center) ;
  • Koo, Hyun Jung (Department of Radiology and Research Institute of Radiology, Asan Medical Center) ;
  • Song, Jae-Kwan (Division of Cardiology, Cardiothoracic Imaging Center, Asan Medical Center) ;
  • Song, Joo Seon (Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Kang, Joon-Won (Department of Radiology and Research Institute of Radiology, Asan Medical Center) ;
  • Yang, Dong Hyun (Department of Radiology and Research Institute of Radiology, Asan Medical Center)
  • 투고 : 2021.05.07
  • 심사 : 2021.08.23
  • 발행 : 2021.12.30

초록

Behçet's disease is a chronic inflammatory disorder involving vessels of various sizes and organs, including the skin, joints, gastrointestinal tract, lungs, and cardiovascular system. The etiology of Behçet's disease is unclear, and clinical diagnosis is important in the absence of definitive laboratory or pathological findings diagnostic of Behçet's disease. Cardiac involvement is rare but might present as endocarditis, myocarditis, pericarditis, or intracardiac thrombosis. This report presents a case of Behçet's disease involving the heart in a 22-year-old man with unusual manifestations of right ventricular fibrosis and intracardiac thrombosis. Cardiac magnetic resonance imaging revealed multiple intracardiac thrombi and delayed diffuse subendocardial enhancement involving the right ventricle. No peripheral eosinophilia was detected. Endomyocardial biopsy showed mixed inflammatory cell infiltrates. Based on the patient's clinical history of oral ulcer and arthritis, a diagnosis of Behçet's disease was made considering the clinical, radiological, and histological findings. Intracardiac thrombi and endomyocardial fibrosis are rare manifestations of Behçet's disease, and the diagnosis is often a clinical challenge. Early diagnosis is important for appropriate management. Behçet's disease should be considered in the differential diagnosis of patients with intracardiac thrombosis and endomyocardial fibrosis of the right chamber.

키워드

참고문헌

  1. Escudier M, Bagan J, Scully C. Number VII Behcet's disease (Adamantiades syndrome). Oral Dis 2006;12:78-84 https://doi.org/10.1111/j.1601-0825.2005.01144.x
  2. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med 1999;341:1284-1291 https://doi.org/10.1056/NEJM199910213411707
  3. Lee I, Park S, Hwang I, et al. Cardiac Behcet disease presenting as aortic valvulitis/aortitis or right heart inflammatory mass: a clinicopathologic study of 12 cases. Am J Surg Pathol 2008;32:390-398 https://doi.org/10.1097/PAS.0b013e31814b23da
  4. Geri G, Wechsler B, Thi Huong DL, et al. Spectrum of cardiac lesions in Behcet disease: a series of 52 patients and review of the literature. Medicine (Baltimore) 2012;91:25-34 https://doi.org/10.1097/md.0b013e3182428f49
  5. 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;28:338-347 https://doi.org/10.1111/jdv.12107
  6. Fernandez-Bello I, Lopez-Longo FJ, Arias-Salgado EG, Jimenez-Yuste V, Butta NV. Behcet's disease: new insight into the relationship between procoagulant state, endothelial activation/damage and disease activity. Orphanet J Rare Dis 2013;8:81 https://doi.org/10.1186/1750-1172-8-81
  7. La Regina M, Orlandini F, Prisco D, Dentali F. Homocysteine in vascular Behcet disease: a meta-analysis. Arterioscler Thromb Vasc Biol 2010;30:2067-2074 https://doi.org/10.1161/ATVBAHA.110.207068
  8. Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behcet's disease: a systematic review. Chest 2000;118:479-487 https://doi.org/10.1378/chest.118.2.479
  9. Davatchi F, Shahram F, Chams-Davatchi C, et al. Behcet's disease: from East to West. Clin Rheumatol 2010;29:823-833 https://doi.org/10.1007/s10067-010-1430-6
  10. Ahn JK, Lee YS, Jeon CH, Koh EM, Cha HS. Treatment of venous thrombosis associated with Behcet's disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol 2008;27:201-205 https://doi.org/10.1007/s10067-007-0685-z