Ischemic Stroke and Myocarditis Revealing Behçet’s Disease in a Young Adult: Diagnostic Challenges and Therapeutic Perspectives
Main Article Content
Abstract
Introduction: Behçet’s disease is a rare, systemic, inflammatory condition that primarily affects young adults. It is characterized by a variety of clinical manifestations. However, neurological and cardiac presentations remain uncommon and often delayed in diagnosis. This disease can lead to severe complications, such as ischemic strokes and myocarditis, highlighting the systemic and complex nature of the condition.
Case presentation: A 27-year-old patient was hospitalized after experiencing an ischemic stroke and myocarditis, which revealed Behçet’s disease. He had a history of oral and cutaneous ulcers, without a prior diagnosis of Behçet. Upon admission, brain imaging confirmed an ischemic stroke, and echocardiography and cardiac MRI showed acute myocarditis. Biological tests confirmed elevated systemic inflammation, which guided the treatment plan. The initial treatment included corticosteroids, immunosuppressors (azathioprine), and cardioprotective therapy. The patient showed significant clinical improvements, although mild deficits persist.
Discussion: Myocarditis in Behçet’s disease is a rare but severe manifestation resulting from inflammation of the heart walls, often associated with other systemic vascular involvement. Although less common than oral or cutaneous ulcers, myocarditis can lead to acute heart dysfunction and even heart failure if not treated promptly. It is generally caused by an excessive inflammatory response, often associated with immune system activation, which affects the coronary circulation and damages the cardiac muscle. Treatment for myocarditis in this context relies on high-dose corticosteroids to control inflammation, followed by long-term immunosuppressive medications like azathioprine. While the initial treatment often leads to a rapid improvement in cardiac function, the risk of long-term complications, such as dilated cardiomyopathy or heart failure, remains high. Close follow-up is therefore essential to prevent these complications and optimize the long-term cardiac prognosis of patients with this rare disease.
Conclusion: The progression of myocarditis in Behçet’s disease can be favorable if diagnosed and treated early, with significant improvement in cardiac function achieved through the use of corticosteroids and immunosuppressive therapy. However, the long-term prognosis remains uncertain due to the risk of chronic cardiac complications, such as dilated cardiomyopathy or heart failure.
Article Details
Copyright (c) 2025 Muhammed OS, et al.

This work is licensed under a Creative Commons Attribution 4.0 International License.
The Journal of Cardiology and Cardiovascular Medicine is committed in making it easier for people to share and build upon the work of others while maintaining consistency with the rules of copyright. In order to use the Open Access paradigm to the maximum extent in true terms as free of charge online access along with usage right, we grant usage rights through the use of specific Creative Commons license.
License: Copyright © 2017 - 2025 | Open Access by Journal of Cardiology and Cardiovascular Medicine is licensed under a Creative Commons Attribution 4.0 International License. Based on a work at Heighten Science Publications Inc.
With this license, the authors are allowed that after publishing with the journal, they can share their research by posting a free draft copy of their article to any repository or website.
Compliance 'CC BY' license helps in:
Permission to read and download | ✓ |
Permission to display in a repository | ✓ |
Permission to translate | ✓ |
Commercial uses of manuscript | ✓ |
'CC' stands for Creative Commons license. 'BY' symbolizes that users have provided attribution to the creator that the published manuscripts can be used or shared. This license allows for redistribution, commercial and non-commercial, as long as it is passed along unchanged and in whole, with credit to the author.
Please take in notification that Creative Commons user licenses are non-revocable. We recommend authors to check if their funding body requires a specific license.
Behçet’s Syndrome – Vasculitis Foundation [Internet]. [cited 2024 Nov 12]. Available from: https://vasculitisfoundation.org/education/vasculitis-types/behcets-syndrome/
Feigenbaum A. Description of Behçet’s syndrome in the Hippocratic third book of endemic diseases. Br J Ophthalmol. 1956 Jun;40(6):355-7. Available from: https://doi.org/10.1136/bjo.40.6.355
Adeeb F, Stack AG, Fraser AD. Knitting the threads of silk through time: Behçet’s disease—Past, present, and future. Int J Rheumatol. 2017 Sep 10;2017:2160610. Available from: https://doi.org/10.1155/2017/2160610
International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). The International Criteria for Behçet’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(12):1745-52. Available from: https://onlinelibrary.wiley.com/doi/10.1111/jdv.12107
International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335(8697):1078-80. Available from: https://pubmed.ncbi.nlm.nih.gov/1970380/
Lavalle S, Caruso S, Foti R, Gagliano C, Cocuzza S, La Via L, et al. Behçet’s disease, pathogenesis, clinical features, and treatment approaches: A comprehensive review. Medicina (Mex). 2024;60(4):562. Available from: https://doi.org/10.3390/medicina60040562
Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77(6):808-18. Available from: https://doi.org/10.1136/annrheumdis-2018-213225
Alibaz-Oner F, Direskeneli H. Advances in the treatment of Behçet’s disease. Curr Rheumatol Rep. 2021;23(6):47. Available from: https://doi.org/10.1007/s11926-021-01011-z
van der Houwen TB, van Hagen PM, van Laar JAM. Immunopathogenesis of Behçet’s disease and treatment modalities. Semin Arthritis Rheum. 2022;52:151956. Available from: https://doi.org/10.1016/j.semarthrit.2022.151956
Ambrose N, Khan E, Ravindran R, Lightstone L, Abraham S, Botto M, et al. The exaggerated inflammatory response in Behçet’s syndrome: identification of dysfunctional post-transcriptional regulation of the IFN-γ/CXCL10 IP-10 pathway. Clin Exp Immunol. 2015;181(3):427-35. Available from: https://doi.org/10.1111/cei.12655
Hu D, Guan JL. The roles of immune cells in Behçet’s disease. Adv Rheumatol. 2023;63(1):49. Available from: https://doi.org/10.1186/s42358-023-00328-w
Emmi G, Becatti M, Bettiol A, Hatemi G, Prisco D, Fiorillo C. Behçet’s syndrome as a model of thrombo-inflammation: The role of neutrophils. Front Immunol [Internet]. 2019 May 14 [cited 2024 Nov 14];10. Available from: https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2019.01085/full
Bettiol A, Alibaz-Oner F, Direskeneli H, Hatemi G, Saadoun D, Seyahi E, et al. Vascular Behçet syndrome: from pathogenesis to treatment. Nat Rev Rheumatol. 2023;19(2):111-26. Available from: https://doi.org/10.1038/s41584-022-00880-7
Atalar E, Erten S, Dogan I, Konak HE. Vascular involvement in Behçet’s disease: An evaluation of 147 cases and literature review. SiSli Etfal Hastan Tip Bul Med Bull Sisli Hosp. 2023;380-6. Available from: https://doi.org/10.14744/semb.2023.89083
Becatti M, Emmi G, Bettiol A, Silvestri E, Di Scala G, Taddei N, et al. Behçet’s syndrome as a tool to dissect the mechanisms of thrombo-inflammation: clinical and pathogenetic aspects. Clin Exp Immunol. 2019;195(3):322-33. Available from: https://doi.org/10.1111/cei.13243
Caforio ALP, Adler Y, Agostini C, Allanore Y, Anastasakis A, Arad M, et al. Diagnosis and management of myocardial involvement in systemic immune-mediated diseases: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Disease. Eur Heart J. 2017;38(35):2649-62. Available from: https://doi.org/10.1093/eurheartj/ehx321
Ammirati E, Frigerio M, Adler ED, Basso C, Birnie DH, Brambatti M, et al. Management of acute myocarditis and chronic inflammatory cardiomyopathy. Circ Heart Fail. 2020;13(11):e007405. Available from: https://doi.org/10.1161/circheartfailure.120.007405
de Roos A. The many faces of myocarditis: Role of cardiac MRI. Radiology. 2022;302(1):70-1. Available from: https://doi.org/10.1148/radiol.2021212121
Parsaei A, Moradi S, Masoumi M, Davatchi F, Najafi A, Kooshki AM, et al. Predictive value of erythrocyte sedimentation rate and C-reactive protein in Behçet’s disease activity and manifestations: A cross-sectional study. BMC Rheumatol. 2022 Feb 11;6:9. Available from: https://doi.org/10.1186/s41927-021-00241-z
Sota J, Capuano A, Emmi G, Iannone F, Cantarini L, Hatemi G, et al. Therapeutic approach to central nervous system involvement of Behçet’s disease. Semin Arthritis Rheum. 2023;61:152206. Available from: https://doi.org/10.1016/j.semarthrit.2023.152206
Moura A, Saraiva M, Costa JM, Domingues K, Martins V. Recurrent myocarditis in the context of Behçet’s disease: a case report. Eur Heart J Case Rep. 2021;5(7):ytab212. Available from: https://doi.org/10.1093/ehjcr/ytab212
Bohbot Y, Pezel T, Demirkıran A, Androulakis E, Houshmand G, Szabo L, et al. European Association of Cardiovascular Imaging survey on cardiovascular multimodality imaging in acute myocarditis. Eur Heart J Cardiovasc Imaging. 2024;25(7):892-900. Available from: https://doi.org/10.1093/ehjci/jeae092
Bohbot Y, Pezel T. Acute myocarditis: An urgent need for evidence-based recommendations. Arch Cardiovasc Dis. 2024;117(6):379-81. Available from: https://doi.org/10.1016/j.acvd.2024.05.116
Myocarditis Organism-Specific Therapy: Specific Organisms and Therapeutic Regimens. 2024 Oct 31 [cited 2024 Nov 16]. Available from: https://emedicine.medscape.com/article/2012214-overview?form=fpf
Montera MW, Mesquita ET, Colafranceschi AS, Oliveira Jr. AC de, Rabischoffsky A, Ianni BM, et al. Brazilian guidelines on myocarditis and pericarditis. Arq Bras Cardiol. 2013;100:01-36. Available from: https://doi.org/10.5935/abc.2013s004
Zhu X, Wang Z, Ferrari MW, Ferrari-Kuehne K, Bulter J, Xu X, et al. Anticoagulation in cardiomyopathy: unravelling the hidden threat and challenging the threat individually. ESC Heart Fail. 2021;8(6):4737-50. Available from: https://doi.org/10.1002/ehf2.13597
Borhani-Haghighi A, Kardeh B, Banerjee S, Yadollahikhales G, Safari A, Sahraian MA, et al. Neuro-Behçet’s disease: An update on diagnosis, differential diagnoses, and treatment. Mult Scler Relat Disord. 2020;39:101906. Available from: https://doi.org/10.1016/j.msard.2019.101906
Alpsoy E, Leccese P, Emmi G, Ohno S. Treatment of Behçet’s disease: An algorithmic multidisciplinary approach. Front Med [Internet]. 2021 [cited 2024 Nov 16];8. Available from: https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.624795/full
Van Der Houwen T, Van Laar J. Behçet’s disease, and the role of TNF-α and TNF-α blockers. Int J Mol Sci. 2020;21(9):3072. Available from: https://doi.org/10.3390/ijms21093072
Fazaa A, Makhlouf Y, Ben Massoud F, Miladi S, Boussaa H, Ouenniche K, et al. Behçet disease: epidemiology, classification criteria, and treatment modalities. Expert Rev Clin Immunol. 2024;14:1437-1448. Available from: https://doi.org/10.1080/1744666X.2024.2388693