A case of Behcet disease with pulmonary artery pseudoaneurysm: long term follow-up

Abstract

Behcet disease (BD) is a chronic inflammatory disorder of unknown etiology with a clinical spectrum that has greatly expanded since it was first described in 1937 by Hulusi Behcet as a triple complex of recurrent oral and genital ulcers and uveitis [1]. Although BD has a worldwide distribution, it is mainly seen in Far East and Middle East countries [2,3]. The highest prevalence is reported from Turkey, 80–370 per 100 000, and ranges from 2 to 30 cases per 100 000 in other Asian countries (16.7 per 100 000 in the Islamic Republic of Iran) [2,3]. The age of disease onset is usually in the second or third decade of life and the male to female ratio is reported to be almost equal. However, the disease runs a more severe course in men and in those with onset before 25 years of age [2]. Since BD does not have pathognomonic symptoms or laboratory findings; diagnosis is made on the basis of the criteria proposed by the International Study Group for Behcet disease in 1990 [4]. According to the criteria, recurrent oral ulceration must be present along with at least 2 of the following: recurrent genital ulceration, eye lesions, skin lesions, a positive pathergy test [2,4,5]. Many disorders, such as cutaneous small vessel vasculitis, inflammatory eye disease, neurological disease, vascular disease, arthritis, are associated with the presence of BD [6]. Pathological features such as smallor large-vessel vasculitis lead to occlusion of the vessels or aneurysm formation [7]. Differential diagnosis of Behcet-induced vasculitis has been described by Kreuger, Hoffman and Merton [8]. More than 200 cases of BD with pulmonary involvement have been reported in the literature. The pulmonary arteries are the second most common site of arterial involvement after the aorta. Aneurysms are more common than thrombosis [2]. Aneurysms associated with BD tend to be multiple and pulmonary artery aneurysms are relatively common, with an associated rate of 1%–10% of patients with BD [9]. Haemoptysis of varying degrees (up to 500 mL) is the most common and predominant symptom [2]. Medical treatment is more beneficial when given in the early stage of the disease [10]. Surgical repair of pulmonary artery aneurysm in BD carries a high risk, with high rates of morbidity and mortality [11]. Recently, endovascular management has been attempted for treatment of vascular involvement. However, when aneurysms are multiple, endovascular treatment is still difficult and challenging. To our knowledge, there is no report on a patient with 4 or more arterial aneurysms treated solely by endovascular management [12,13].