Intracardiac Thrombi in Behçet's Disease: Systematic Review of 218 Patients' Clinical Characteristics and Treatments.

BACKGROUND

Behçet's disease (BD) is a rare autoimmune disorder with significant cardiovascular complications, including intracardiac thrombosis (ICT), which can lead to high morbidity and mortality.

METHODS

We conducted a comprehensive literature search across multiple databases up to December 2024, identifying articles related to BD and ICT. Data were extracted from case reports and cohort studies, focusing on demographics, clinical presentations and treatment outcomes.

RESULTS

A total of 218 cases of ICT in BD were analysed, predominantly affecting young males from regions along the historical Silk Road. ICT often presented as the initial manifestation of BD (53.21% of cases), with fever, dyspnoea and haemoptysis as common symptoms. The right heart chambers were most frequently involved. Treatment primarily involved corticosteroids, immunosuppressants and anticoagulants, with a complete resolution rate of 67.22% (n=160). Surgical interventions were associated with high recurrence and mortality rates of 60% and 34.78%, respectively.

CONCLUSION

ICT in BD is a serious complication that requires prompt recognition and management. A multimodal treatment approach combining corticosteroids, immunosuppressants and anticoagulants is recommended to improve outcomes. Surgical intervention carries a higher risk of mortality and morbidity, making it a less preferred treatment option. Clinicians should maintain a high index of suspicion for ICT in BD patients and initiate timely medical therapy to mitigate complications. Further studies are needed to optimise treatment strategies and explore long-term outcomes.