THU0538 Three Behçet's Disease Patients with Pulmonary Artery Aneurysm
Authors: Z.S. Arıcı, F. Kara-Eroglu, E.D. Batu, N. Besbas, T. Hazırolan, A. Düzova, S. Ozen
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background</h3> Behçet9s disease (BD) is most common cause of pulmonary artery aneurysms (PAAs) which is one of the characteristic lesions of BD. PAA is associated with the highest mortality in BD. Herein we report three effectively treated patients with PAA in Behcet9s disease. <h3>Objectives</h3> Cases reports: The first patient was a 14-year-old boy admitted to our clinic with complaints of painful nodules on his leg, recurrent oral and genital ulcers. On his fourth year follow-up, when he was admitted with hemoptysis, computerized tomography angiography (CTA) showed PAAs. Successful embolization of a giant PAA was performed. He received pulse and oral steroid therapy and six pulses of i.v. cyclophosphamide. After suppression of the disease he underwent lower lobectomy for the right lung. He is now well, off immunosuppressive and has recently graduated from university. The second case, a 16-year-old boy presented with hemoptysis. He was previously diagnosed as Budd-Chiari syndrome with a thrombus completely obliterating vena cava inferior and hepatomegaly. Pathergy test was negative. There were no oral ulcers but on examination he had a scrotal ulcer. HLA-B51 was positive. CTA showed aneurysmatic distentions and thrombus in distal parts of bilateral pulmonary arteries. He responded to pulse and oral steroid therapy, six pulses of i.v. cyclophosphamide, and interferon-alpha for 6 months. One year after presentation, thoracal magnetic resonance angiography showed normal pulmonary arteries. The third patient was a 17-year-old boy who had underwent surgery for intracardiac thrombus and was referred due to recurrent right ventricular (RV) thrombus. He had complaints of fever, cough and weight loss. He described frequent oral ulcers. HLA B51 was positive. Pathergy test was negative. CTA revealed bilateral pulmonary artery thrombosis, right PAA and a mass configuration in RV. He was given pulse and oral steroid, six pulse of i.v. cyclophosphamide and interferon-alpha for 6 months. One month after steroid and cyclophosphamide treatment CTA showed normal pulmonary arteries. Four months after discharge, echocardiogram showed resolution of the intracardiac thrombus. <h3>Conclusions</h3> The latter two patients highlight the inadequacy of the existing criteria in diagnosing BD, since neither met the criteria. We suggest that it is important to consider the diagnosis of BD in similar patients since early recognition and intensive use of immunosuppressives improves the prognosis in patients with PAA. <h3>References</h3> Agha A, Bella AM, Assiri AH, Al-Hakami M. Can Behcet9s Disease Related Pulmonary Arterial Aneurysms be Completely Resolved? Open Rheumatol J. 2011;5:88-90. Ozen S, Bilginer Y, Besbas N, Ayaz NA, Bakkaloglu A. Behçet disease: treatment of vascular involvement in children. Eur J Pediatr. 2010;169:427-30. Farouk H. Behçet9s disease, echocardiographers, and cardiac surgeons: together is better. Echocardiography. 2014;31:783-7. Hamuryudan V, Yurdakul S, Moral F, Numan F, Tüzün H, Tüzüner N, Mat C, Tüzün Y, Ozyazgan Y, Yazïci H. Pulmonary arterial aneurysms in Behçet9s syndrome: a report of 24 cases. Br J Rheumatol. 1994;33:48-51. Ozguler Y, Hatemi G, Yazici H. Management of Behçet9s syndrome. Curr Opin Rheumatol. 2014;26:285-91. <h3>Disclosure of Interest</h3> None declared