SAT0493 THE CHALLENGE OF TREATİNG PULMONARY VASCULITIS IN BEHÇET’S DISEASE: TWO PEDIATRIC CASES
Authors: Selcan Demir, Erdal Sag, Ummusen Kaya Akca, Tuncay Hazirolan, Yelda Bilginer, Seza Özen
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Behçet's Disease (BD) is a multisystemic autoinflammatory disease and the most severe complication of BD is pulmonary artery involvement (PAI). Data regarding treatment and outcomes of pediatric patients with PAI is very limited. <h3>Objectives:</h3> Herein, we report two pediatric patients with BD presenting with PAI and treated successfully with aggressive immunosuppressive treatment. <h3>Methods:</h3> Demographic data, clinical manifestations, laboratory and radiological findings, and treatments of patients were documented from patient charts retrospectively. <h3>Results:</h3> <h3>Case 1:</h3> A 15-year-old boy was admitted with abdominal pain and fever. An abdominal Doppler ultrasonography (USG) showed stenosis of vena cava inferior (VCI) with a thrombus. Transthoracic echocardiography (TTE) detected that the thrombus extended from VCI to the right atrium. When he started to have hemoptysis, he was referred to our hospital. TTE showed a mass in RV. The computed chest and abdominal tomography angiography (CTA) showed bilateral aneurysmatic dilatation with thrombi in the pulmonary arteries and thrombosis in vena hepatica. The pathergy test was negative and the HLA B5 was negative. According to the ICBD, the patient had been diagnosed as BD due to genital ulcers and vascular involvement. He was given pulse methylprednisolone (MP) 500 mg 3 days along and followed by oral prednisolone 1 mg/kg/day, intravenous cyclophosphamide at a dose of 15 mg/kg every 3 weeks for a total of 6 cycles, and Interferon-α2a (IFN-α2a) 3 times a week. Within one month, hemoptysis and fever disappeared, and CRP values normalized. After a three-month treatment, TTE and CTA revealed that thrombi shrank significantly. The dosage of prednisolone was tapered gradually and stopped 2 years later. Immunosuppressive treatment was continued with adalimumab. The patient has been followed in remission for nearly 6 years. <h3>Case 2:</h3> A fifteen-year-old boy was referred to our hospital for the evaluation of fever for over 4 months and a thrombus in his right ventricle. He had a medical history with cough, fever, intermittent hemoptysis and weight loss for the past 3 months. Physical examination revealed acne-like rashes over face and back, oral ulcers. A CTA confirmed the thrombus in RV and showed bilateral multiple aneurysms along the pulmonary artery and its branches. According to ICBD, the patient was diagnosed with BD due to having aphthous ulcers, pseudofolliculitis, and vascular involvement. Iv MP (500 mg/day) for 3 days was followed by oral prednisolone 1 mg/kg/day, which was subsequently tapered. Iv cyclophosphamide at a dose of 500 mg was also given every 3 weeks for a total of 6 cycles, followed by oral azathioprine (AZA). Concomitant subcutaneous IFN-α2a was given two times per week for 6 months. Within two weeks, cough and fever disappeared, CRP values normalized. After 1 year the pulmonary artery aneurysm disappeared and cardiac thrombosis resolved. We have been following the patient with AZA for four years without recurrence. <h3>Conclusion:</h3> We present two pediatric patients with pulmonary involvement of BD. PAI is a life-threatening condition and should be managed with more aggressive medical therapy. Early diagnosis and aggressive immunosuppressive treatment are very important in PAI. We strengthened our treatment with IFN-α2a. There is no data in the literature regarding the use of IFN-α-2a in PAI treatment along with low dose cyclophosphamide. There were no mortality or recurrences within the 6 and 4 years follow up period. An aggressive immunosuppressive therapy leads to better prognosis in this most dreadful complication of BD. <h3>References</h3> [1] Ozen S, Bilginer Y, Besbas N, Ayaz NA, Bakkaloglu A. Behcet disease: treatment of vascular involvement in children. Eur J Pediatr. 2010;169(4):427-30. <h3>Disclosure of Interests:</h3> Selcan Demir: None declared, Erdal Sag: None declared, Ummusen Kaya Akca: None declared, Tuncay Hazirolan: None declared, Yelda Bilginer: None declared, Seza Özen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche