¹Hiba Ibrahim Khogali, ¹Aseed Mestarihi, ¹Walaaeldin Elkhalifa

¹Tawam Hospital

Background(s): Bechet’s disease is a complex, chronic inflammatory disorder that affects multiple systems in a relapsing-remitting status. The exact etiology remains unknown, but the hallmark of the disease is arterial/venous vasculitis manifestation. It is diagnosed mainly clinically, which adds more challenges in management and treatment. The diverse range of symptoms in Bechet’s disease underscores its diagnostic complexity. We are reporting two challenging cases that fulfill the ICBD criteria with potential involvement of the cardiovascular and central nervous systems respectively.

Method(s): 2 case report: Tertiary teaching hospital

Result(s): Case 1: A 43-year-old Emirati male diagnosed with  Bechet disease presented to the ER with chest pain radiating to the left shoulder. ECG revealed an inferolateral myocardial infarction (MI). Cardiac catheterization identified an ectatic lesion in RCA and LCA, which was managed through stenting. Despite successful PCI, the patient continued to experience recurring atypical chest pain without ECG findings nor elevation of cardiac markers for a year. A subsequent PCI documented occlusion of  RCA and the presence of multiple coronary luminal irregularities of the LAD and the RCA, suggesting active vasculitis. The patient commenced on RTX  infusion followed by  AZA and maintained at remission.

Case 2: A 23-year-old Sudanese male, presented to the ER with a one-week history of severe headache, fever, and blurred vision. Prior to this presentation, the patient declared to have four episodes of red painful eyes, multiple painful mouth ulcers, and two attacks of genital ulcers in the past year. Brain MRA was negative, however, MRV showed a large clot in the right sigmoid sinus and internal jugular vein suggestive of sinus thrombosis. Antibiotics and anticoagulants were initiated, but there was no improvement. Based on the above clinical manifestation and positive neuro-radiological findings, a diagnosis of acute neurobehect disease was established. Pulse steroids regime followed by infliximab infusions resulted in significant improvement.

Conclusion(s): Vascular manifestation of Behect disease can be devastating with a fatal outcome if not diagnosed early with prompt management. Additional research is required to enhance our understanding of the underlying pathophysiology and optimize management strategies for Bechet’s disease