[Intracardiac thrombosis in Behçet disease: clinical presentation and outcome of three cases].

BACKGROUND

Behçet's disease is recognized as a multisystem disorder that affects mainly young adults in Mediterranean, Middle Eastern, and Far Eastern countries. The diagnosis is very difficult because there is no laboratory test. Clinical features - such as orogenital aphtae, ocular and skin lesions, arthritis, and neurologic, gastrointestinal, vascular, and pulmonary symptoms - are helpful for diagnosis. Various cardiovascular manifestations, such as pancarditis, acute myocardial infarction, conduction system disturbances, and valvular diseases, have been reported but are rare. Intracardiac thrombus formation, as seen in our patients, is exceptional even among cardiovascular cases of Behçet's.

OBSERVATIONS: We report three cases of intracardiac thrombosis among 204 patients followed for Behçet's disease within our unit over a period of 7 years. We report outcomes after corticosteroid, cyclophosphamide, and oral anticoagulant therapy.

DISCUSSION: Cardiovascular involvement has been reported in 7 % to 29 % of patients with Behçet's syndrome. Intracardiac thrombosis is extremely rare and the right heart is the most common site of involvement. The first symptoms and signs of the disease frequently precede systemic organ manifestations.

CONCLUSION

Diagnosis of Behçet's disease might be considered if a patient presents with a mass in the right-sided cardiac chambers, even in the absence of the characteristic clinical features of the condition. This is particularly applicable if the patient is a young male from the Mediterranean basin or the Middle East. We suggest that the treatment could include colchicine, anticoagulant therapy, and corticosteroids and discuss immunosuppressive therapy.