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Resolution of Behçet's Syndrome Associated Pulmonary Arterial Aneurysms with Infliximab

https://doi.org/10.1016/j.semarthrit.2011.02.006Get rights and content

Introduction

We describe the successful treatment of pulmonary arterial aneurysms in Behçet's syndrome using a tumor necrosis factor (TNF) inhibitor.

Methods

A case is reported of Behçet's syndrome complicated by pulmonary arterial aneurysms that responded to anti-TNF therapy. This is accompanied by a literature review of previously published cases. We searched the English language medical literature using the PubMed and Medline search terms: “Behçet's,” “Pulmonary aneurysms,” and “infliximab,” “etanercept,” or “adalimumab.”

Results

A 43-year-old man with a 6-month history of oral and genital ulcers, weight loss, and fatigue developed arterial aneurysms in the common carotid and common iliac arteries and thromboses in a femoral vein and pulmonary arteries. Treatment with high-dose oral corticosteroids and pulsed intravenous cyclophosphamide was initiated but while on treatment he developed pulmonary arterial aneurysms with hemoptysis. His treatment was changed to intravenous infliximab with methotrexate to which he showed a good response with marked clinical improvement, reduction in his inflammatory markers, and regression of the pulmonary arterial aneurysms. The review of the literature identified 3 reported cases of treatment of pulmonary arterial aneurysms in Behçet's syndrome with anti-TNF therapy, with good outcomes in each case.

Conclusions

Pulmonary artery aneurysms are important complications of Behçet's syndrome. Anti-TNF inhibitors should be considered in patients who do not respond to treatment with corticosteroids and cyclophosphamide.

Section snippets

Methods

The patient described in the case report was referred to the Behçet's clinic at Hammersmith Hospital, London, where he underwent further investigation and treatment. Information, including operation notes and histopathology, was obtained from the referring hospital. Clinical and laboratory information was collected from his medical records. We reviewed the English language medical literature using the PubMed and Medline search terms “Behçet” and “pulmonary arterial aneurysms” together with

Results

A 43-year-old unemployed British white man presented to his local hospital with an enlarging right-sided neck lump and a pulsatile “barking” sound in his right ear. Ultrasonography showed a saccular aneurysm of the right common carotid artery. The carotid artery was resected and continuity was restored with autologous great saphenous vein interposition graft. On histology, the adventitia of the excised artery showed inflammation around the vasa vasorum and extravasation of red cells (Fig. 1).

Discussion

Vascular involvement is common in BS, having been reported in 14% of patients in Turkey (3) and 25% in the UK (4, 5). It is more common in men than in women. In our own experience we have found vascular complications in 21% of men and 14% of women, satisfying the International Study Group criteria (2). We present here a case of aggressive BS with multiple aneurysms and thromboses. The association of deep vein thromboses with pulmonary arterial aneurysms, as seen in our patient, has been

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    DOH was in receipt of funding from the British Heart Foundation. The study was made possible by funding from the National Institute of Research Comprehensive Biomedical Research Centre at Imperial College Healthcare NHS Trust.

    The authors have no conflicts of interest to disclose.

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