Available online 20 April 2024, 107648

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Behçet's can cause valvulitis, aortic issues, and pseudoaneurysm.

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Behçet's valvulitis mimics infective endocarditis, but lacks bacteria.

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Multidisciplinary care improves Behçet's outcomes.

Abstract

This case report presents a 20-year-old male patient initially diagnosed with infective endocarditis, later correctly identified as Behçet's syndrome. The patient's complex clinical presentation, including chest pain, aortic dilation, severe aortic regurgitation, and aortic root abscess, posed significant diagnostic and therapeutic challenges. Despite initial misdiagnosis and treatment difficulties, the patient's condition significantly improved with appropriate immunosuppressive therapy, underscoring the potential for successful management of this complex condition. This case serves as a valuable reminder of the diagnostic challenges posed by Behçet's syndrome and the importance of considering this condition in patients presenting with symptoms suggestive of infective endocarditis.

Section snippetsINTRODUCTION

Behçet's syndrome is a rare autoimmune disorder characterized by recurrent oral and genital ulcers, along with various systemic manifestations, including cardiovascular involvement [1]. While cardiac manifestations are less common, they can lead to serious complications such as endocarditis, valvular regurgitation, and aortic aneurysm. Due to its rarity and diverse clinical presentation, diagnosing Behçet's syndrome-related cardiac involvement can be challenging, especially when presenting with

CASE REPORT

A 20-year-old male patient was admitted to the hospital due to two weeks of chest pain. Echocardiography revealed dilation of the ascending aorta and aortic arch, along with severe aortic regurgitation. Enhanced computed tomography scan indicated aortic aneurysm (max diameter: 50.2mm), pseudoaneurysm and dissection at the aortic sinus(Fig. 1 A). The patient underwent Wheat's surgery (Wheat's surgery refers to the aortic valve and ascending aorta replacement surgery that preserves the aortic

DISCUSSION

Behçet's syndrome is an inflammatory condition affecting multiple organ systems characterized by recurrent oral and genital ulcers. This case meets the diagnostic criteria for Behçet's syndrome according to the 2014 Behçet's syndrome guidelines, the International Criteria for Behcet's Disease (point score system) where a score of 4 indicates a diagnosis of Behcet's Disease (Ocular lesions 2, Genital aphthosis 2, Oral aphthosis 2, Skin lesions 1, Neurological manifestations 1, Vascular

CONCLUSION

Behçet's syndrome involved in cardiovascular system can present as conditions such as valvulitis, aortic dissection, aortic aneurysm, and pseudoaneurysm. We emphasized the importance of multidisciplinary management and standardized treatment protocols for Behçet's syndrome, which can effectively improve the prognosis of critically ill patients.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

CRediT authorship contribution statement

Wei Qu: Writing – review & editing, Writing – original draft, Investigation. Youping Chen: Writing – review & editing, Writing – original draft, Supervision. Zhenlu Zhang: Writing – review & editing, Supervision.

Declaration of competing interest

none.

Acknowledgments

None.

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