Emel ISIYEL, Sevcan BAKKALOĞLU, Deniz OĞUZ, İdil YENİCESU, Öznur BOYUNAĞA, Yeşim ÖZDEMİR, Çağrı DAMAR, Yaşar KANDUR, Meltem AKÇABOY, Ayşe Tana ASLAN, Tuğba ŞİŞMANLAR, Enver HASANOĞLU, Necla BUYAN

An adolescent case of extensive Behçet’s disease successfully treated with Infliximab

Cardiac involvement is an uncommon and life-threatening complication ofBehçet’s Disease. We present a 14-year-old boy, admitted to our hospital forrecurrent hemoptysis. In his radiologic evaluation, a right ventricular thrombusand pulmonary arterial aneurysm were identified. He was diagnosed withBehçet’s Disease, and then he received prednisone and cyclophosphamide.However, his cardiac thrombus enlargened. After his treatment was replacedwith infliximab, the pulmonary aneurysms regressed, and the cardiac thrombusdisappeared. In conclusion, infliximab should be considered as a reliableoption for vascular Behçet’s Disease resistant to conventional treatment.

PDF

___

1. Wirth M, Jellimann JM, Jellimann S, Hascoet JM. Darie C, Knezinsky M, Demolombe-Rague S, et al. Cardiac pseudotumor revealing Behcet's disease. Rev Med Interne 2005; 26: 420-424.

2. Hatemi G, Seyahi E, Fresko I, Talarico R, Hamuryudan V. Behcet's syndrome: A critical digest of the 2014- 2015 literature. Clin Exp Rheumatol 2015; 33(6 Suppl 94): S3-S14.

3. Koné-Paut I, Shahram F, Darce-Bello M, et al; PEDBD Group. Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD. Ann Rheum Dis 2016 ;75: 958-964

4. Koc Y, Gullu I, Akpek G, et al. Vascular involvement in Behcet's disease. J Rheumatol 1992; 19: 402-410.

5. Mutlu S, Scully C. The person behind the eponym: Hulusi Behcet (1889-1948). J Oral Pathol Med 1994; 23: 289-290.

6. Criteria for diagnosis of Behcet's disease. International Study Group for Behcet's Disease. Lancet 1990; 335: 1078-1080.

7. Ames PRJ, Steuer A, Pap A, Denman AM. Thrombosis in Behcet's disease: A retrospective survey from a single UK centre. Rheumatology (Oxford) 2001; 40: 652-655.

8. Louali FE, Tamdy A, Soufiani A, et al. Cardiac thrombosis as manifestation of Behçet syndrome. Tex Heart Inst J 2010; 37: 568-571.

9. Gopathi S, Hurt RT, Guardiola J. Intracardiac thrombus in Behçet’s disease: A rare case in the United States. Respir Med CME 2011; 4: 154-156.

10. Marc K, Iraqui G, Jniene A, Benamor J, Bourkadi JE. Intracardiac thrombus and pulmonary artery aneurysm in Behcet's disease. Rev Mal Respir 2008; 25: 69-72.

11. Noureddine M, Charei N, Drighil A, Chraibi N. Right intracardiac thrombus in Behcet's disease. Arch Mal Coeur Vaiss 2004; 97: 925-928.

12. Aksu T, Tufekcioglu O. Intracardiac thrombus in Behçet's disease: Four new cases and a comprehensive literature review. Rheumatol Int 2015; 35: 1269- 1279.

13. Seyahi E, Melikoglu M, Akman C, et al. Pulmonary artery involvement and associated lung disease in Behçet disease: A series of 47 patients. Medicine (Baltimore) 2012; 9: 35-48.

14. Hammami R, Abid L, Frikha F, et al. Intracardiac thrombus in a young man: Don't forget Behcet's disease! Intern Med 2012; 51: 1865-1867.

15. Demirelli S, Degirmenci H, Inci S, Arisoy A. Cardiac manifestations in Behcet's disease. Intractable Rare Dis Res 2015; 4: 70-75.

16. Hatemi G, Silman A, Bang D, et al. Management of Behcet disease: A systematic literature review for the European League Against Rheumatism evidencebased recommendations for the management of Behcet disease. Ann Rheum Dis 2009; 68: 1528-1534.

17. Endo LM, Rowe SM, Romp RL, Buckmaster MA, Atkinson TP. Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African- American adolescent with oculocutaneous albinism. Clin Rheumatol 2007; 26: 1537-1539.

18. Schreiber BE, Noor N, Juli CF, Haskard DO. Resolution of Behcet's syndrome associated pulmonary arterial aneurysms with infliximab. Semin Arthritis Rheum 2011; 41: 482-487.

19. Yoshida S, Takeuchi T, Yoshikawa A, et al. Good response to infliximab in a patient with deep vein thrombosis associated with Behcet disease. Mod Rheumatol 2012; 22: 791-795.

20. Seyahi E. Behçet's disease: How to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol 2016; 30: 279-295