Right Atrium Thrombus and Pulmonary Artery Aneurysm in a Man with Behcet's Disease

Abstract

A30-year-old man, with no prior pathologic antecedent was admitted to the internal medicine department for a 3 month history of cough and bloody sputum. These symptoms occurred in the context of apyrexia, night sweating and 6 kilogram weight loss. On physical examination the patient appeared pale, BMI was 17 kg/m 2 , crackles were heard in the left lung. The skin examination noted painful genital and oral ulcers with pustlar lesions in the back.Chest X-ray showed left hilar opacity. Angio CT scan revealed an aneurysm of the left inferior pulmonary artery (Picture 1) with parietal thrombus, right basal pulmonary infarction, and right intra-atrial thrombus (Picture 2). Echocardiography noted the mobile thrombus in the right atrium (Picture 3) and another thrombus in the vena cava junction. Laboratory tests found inflammatory anemia. The infectious analysis (VDRL-TPHA, Mycobacteria) was negative. There were definite findings for thrombophilia or connectivitis, however HLA-B51 was positive. The diagnosis of Behçet's disease was made. The patient was treated with cyclophosphamide and methylprednisolone pulses relayed by oral prednisone 1 mg/kg/j and anticoagulation. After 5 months there was disappearance of intracardiac thrombi and marked regression of the pulmonary artery aneurysm.The location is rare in Behçet's disease: from 1 to 5% in clinical series, 16.5% on a register of autopsies in Japan and

Pulmonary artery aneurysms in Behçet disease

“…21 Pulse methylprednisolone followed by oral prednisolone is one routine regimen for Behçet's disease. 48,50,59 Monthly intravenous cyclophosphamide 1 g, and azathioprine 2.5 mg/kg/day may also be employed. 88 Tumor necrosis factor-neutralizing monoclonal antibody infliximab was an effective treatment for a case of potentially lethal PAA in Behçet's disease.…”

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“…This manifestation occurs with predilection in young males and rarely reveals BD. It can be isolated, but must necessarily be investigated for other vascular damage, including Pulmonary Artery Aneurysms (PAA) [4]. It usually occurs as part of an already advanced Behçet's disease and it is rarely inaugural.…”

Behcet's Disease: New Concepts in Cardiovascular Involvements and Future Direction for Treatment

“…Based on autopsy findings, it looks that endomyocardial fibrosis plays a role in the intracardiac thrombus development in some patients [86], but it is not clear whether they are secondary to underlying endocarditis or endomyocardial fibrosis, because in some case reports, a normal underlying myocardium was described [87]. Due to high specificity of right heart thrombus in BD, in any patient with this finding, diagnosis of BD should be considered [86, 88, 89]. Intracardiac thrombus is the major differential diagnosis when a young patient presents with an intracardiac mass [90].…”