Pulmonary embolism due to an intracardiac thrombosis in a patient affected by Behçet’s disease: a case report

TL;DR

A man with Behçet’s disease developed a rare case of pulmonary embolism caused by a heart thrombus, successfully treated with anticoagulation and immunosuppressive drugs.

Contribution

This case report highlights a rare cardiac complication of Behçet’s disease and its successful management with combined therapy.

Findings

Pulmonary embolism in a Behçet’s disease patient was caused by a floating thrombus in the right ventricle.

The thrombus resolved after 5 months of treatment with anticoagulation and immunosuppressive drugs.

Behçet’s disease can cause thrombosis in atypical locations, indicating high systemic inflammation.

Abstract

Behçet’s disease is an inflammatory condition, caused by vasculitis of big and small veins and arteries in which, although vascular inflammation is the basis of disease, cardiac involvement is rare. We present a rare case of a man, affected by Behçet’s disease, with pulmonary embolism due to a floating thrombus in the right ventricle.

We report a case of a 36-year-old man admitted to emergency department due to dyspnoea and haemoptysis. He had already been diagnosed with Behçet’s disease, and he was in therapy with low doses of azathioprine and prednisone from three months. Thorax CT scan detected pulmonary embolism with pulmonary infraction. No evidence of deep vein thrombosis was found. The echocardiogram pointed out a floating mass of at least 30 mm in the right ventricle. Cardiac magnetic resonance confirmed the diagnosis of right ventricle thrombosis. On the hypothesis of an inflammatory genesis of the thrombosis, immunosuppressive drugs and anticoagulation with vitamin K antagonist were prescribed. The patient underwent echocardiograms every 3 weeks, and the mass disappeared 5 months later.

Behçet’s disease is a systemic inflammatory disorder that often affects vessels and rarely the heart. Thrombosis can be the only clinical feature of primary or relapsing events with also atypical origin site. Thrombosis suggests a high inflammatory status that needs to be balanced with the right immunosuppressive therapy, associated to anticoagulation.

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