Diagnostic dilemma: Intracardiac mass in a woman with Behçet's syndrome

Behçet disease (BD) is a chronic multisystem disorder with vasculitis underlying its systemic manifestations. Cardiac involvement and particularly left ventricular intracardiac thrombus are rarely diagnosed in the course of BD and are often associated with poor prognosis. The causes of intracardiac thrombi are unknown. It is plausible that specific proinflammatory pathways resulting in the endothelial cell injury and hypercoagulation contribute to the formation of thrombotic masses in the heart. Known thrombophilic factors such as methylenetetrahydrofolate reductase gene mutations, factor V Leiden mutation, proteins S and C, antithrombin III, activated protein C resistance, and antiphospholipid antibodies may contribute to the formation of intracardiac thrombi in BD. We report a case of a 24-year-old male patient with BD presented with left ventricular thrombus. Transthoracic echocardiography allowed to describe and monitor such a rare cardiac manifestation of the disease. A combination of high-dose corticosteroid and azathioprine successfully dissolved intracardiac thrombus within ten days without anticoagulation.

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Left ventricular intracardiac thrombus in a patient with Behçet disease successfully treated with immunosuppressive agents without anticoagulation: a case report and review of the literature

Лисицына

Alekberova

Ovcharov

et al. 2015

Rheumatol Int

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Imaging manifestations of Behcet’s disease: Key considerations and major features

Mehdipoor

Davatchi

Ghoreishian

et al. 2018

European Journal of Radiology

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Cardiac Inflammatory Pseudotumors in Behçet’s Disease

Bouomrani

Belgacem

2019

JHC

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Introduction Cardiac non-specific inflammatory pseudotumors (NSIPT) are exceptionally associated to Behçet's disease (BD) and represent a real diagnostic and therapeutic challenge. The meaning and the mechanisms of this association are not yet well understood. The purpose of this paper is to study the epidemiologic, therapeutic, and evolutionary characteristics of cardiac NSIPT during BD Methods Systemic review of all reported cases of cardiac NSIPT associated with BD. Results We found only 6 cases of NSIPT associated with BD. Of these six patients, 4 were men (66.66%) and 2 were women (33.33%): Sex ratio =2. The average age was 26.66 years (9-35 years). The pseudotumor was unique in all cases. The chronology of occurrence of these NSIPT compared to the underlying angiitis was variable: inaugural of the disease in 4 cases, and complicating a previously known BD in 2 cases. The surgery was performed in all cases. It was carried out for diagnostic purpose in 4 cases, and therapeutic in the other 2. Additional medical treatment based on systemic corticosteroids with or without immunosuppressants was indicated in 4 patients. The evolution was favorable in 5 cases and a single case was quickly fatal. Recurrence of NSIPT was reported in one patient (20%). Conclusion The results of this review suggest a very likely association between BD and cardiac NSIPT; especially because of the scarcity of these two conditions in the general population, and the epidemiological characteristics clearly different from those of cardiac NSIPTs in the general population. The pathogenic mechanisms common to these two conditions (immune, inflammatory, reactive, and vascular) reinforce this causal link. The main differential diagnoses of these pseudotumors during BD remain cancer and intracardiac thrombosis.

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Diagnostic dilemma: Intracardiac mass in a woman with Behçet's syndrome

Cited by 4 publications

References 4 publications

Left ventricular intracardiac thrombus in a patient with Behçet disease successfully treated with immunosuppressive agents without anticoagulation: a case report and review of the literature

Left ventricular intracardiac thrombus in a patient with Behçet disease successfully treated with immunosuppressive agents without anticoagulation: a case report and review of the literature

Imaging manifestations of Behcet’s disease: Key considerations and major features

Cardiac Inflammatory Pseudotumors in Behçet’s Disease

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