Article Text

PDF
THU0538 Three Behçet's Disease Patients with Pulmonary Artery Aneurysm
  1. Z.S. Arıcı1,
  2. F. Kara-Eroglu1,
  3. E.D. Batu1,
  4. N. Besbas1,
  5. T. Hazırolan2,
  6. A. Düzova1,
  7. S. Ozen1
  1. 1Department of Pediatrics, Division of Rheumatology
  2. 2Department of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey

Abstract

Background Behçet's 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 Behcet's disease.

Objectives 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.

Conclusions 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.

References

  1. Agha A, Bella AM, Assiri AH, Al-Hakami M. Can Behcet's Disease Related Pulmonary Arterial Aneurysms be Completely Resolved? Open Rheumatol J. 2011;5:88-90.

  2. 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.

  3. Farouk H. Behçet's disease, echocardiographers, and cardiac surgeons: together is better. Echocardiography. 2014;31:783-7.

  4. Hamuryudan V, Yurdakul S, Moral F, Numan F, Tüzün H, Tüzüner N, Mat C, Tüzün Y, Ozyazgan Y, Yazïci H. Pulmonary arterial aneurysms in Behçet's syndrome: a report of 24 cases. Br J Rheumatol. 1994;33:48-51.

  5. Ozguler Y, Hatemi G, Yazici H. Management of Behçet's syndrome. Curr Opin Rheumatol. 2014;26:285-91.

Disclosure of Interest None declared

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.