Clin Exp Rheumatol. 2026 Sep 4. doi: 10.55563/clinexprheumatol/6h353p. Online ahead of print.
ABSTRACT
Behçet's disease (BD) is a chronic multisystemic inflammatory vasculitis which can cause cardiac involvement in less than 6%. It can affect all cardiac wall giving rise to pericarditis, myocarditis, endocardial lesions with valvular involvement (especially aortic insufficiency), intracardiac thrombosis, coronary arteritis, myocardial infarcts, arrythmia, and aortitis. Clinical presentation can be confusing, silent, or overshadowed, so diagnosis is frequently delayed. Early diagnosis is mandatory and different imaging techniques play a pivotal role to screen the heart. Biological agents, including tumour necrosis factor inhibitors, show promising results. Cardiac surgery must be accompanied by immunosuppressive therapy to prevent iatrogenic or trauma-triggered pseudo aneurysm secondary to arterial hypersensitivity. A multidisciplinary approach and tailored monitoring are essential to improve patient outcome. Strong data are still lacking to define evidence-based diagnostic algorithms, risk-stratification tools, and standardised management strategies for this severe cardiac manifestation. Besides, further studies are needed to determine specific biomarkers to refine early diagnosis, assess therapeutic efficiency, and prognosis. Through a comprehensive literature review, we aim to synthesise current data on the epidemiology, clinical presentation, contemporary imaging modalities, prognosis, and evidence‑based management strategies of cardiac involvement in BD, and to highlight emerging perspectives.
PMID:42752378 | DOI:10.55563/clinexprheumatol/6h353p