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Behcet Syndrome Treatment & Management

  • Author: C Egla Rabinovich, MD, MPH; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Apr 18, 2016
 

Medical Care

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  • Treatment of Behçet syndrome must be tailored to each patient's clinical manifestations. Corticosteroids are considered palliative; they are useful in controlling acute manifestations, but progression of CNS and ocular disease may occur in patients treated with corticosteroids alone.
  • Cytotoxic medications are usually indicated in patients with ocular, CNS, and vascular disease. Biologic medications are also being used in patients with these complications. Decreasing morbidity and mortality is the goal of treatment for children with Behçet syndrome.
  • The European League Against Rheumatism (EULAR) has recently released guidelines for the management of Behçet disease.[13]
  • There are not drugs targeted or indicated for Behçet syndrome by US regulatory agencies; therefore, the use of medications is considered off-label.
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Surgical Care

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  • Surgical resection of aneurysms with graft placement should be considered if feasible because of the high risk of aneurysmal rupture. However, complications of arterial surgery, such as aneurysms at the surgical site (similar to a pathergylike effect) and local thrombus formation, commonly occur.
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Consultations

A rheumatologist should be consulted for all patients with Behçet syndrome. For children, a pediatric rheumatologist is preferable. Consider other consultations depending on patient signs and symptoms.

  • All patients should have regular eye examinations by an ophthalmologist experienced with vasculitis.
  • Consultation with a neurologist should be considered for patients with CNS symptoms.
  • A consultation with a gastroenterologist is appropriate for evaluation and management of abdominal symptoms.
  • Consultation with a vascular surgeon is important for patients with aneurysm formation.
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Diet

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  • No specific dietary recommendations are needed for patients with Behçet syndrome. However, patients on long-term corticosteroid treatment should avoid excessive weight gain and follow a low-salt, low-fat diet.
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Activity

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  • Restriction of activity should be tailored to a patient's clinical manifestations.
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Contributor Information and Disclosures
Author

C Egla Rabinovich, MD, MPH Associate Professor and Co-Division Chief, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center

C Egla Rabinovich, MD, MPH is a member of the following medical societies: American College of Rheumatology

Disclosure: Received grant/research funds from Abbott for clincal trial; Received grant/research funds from UCB for clinical trial; Received grant/research funds from Janssen for clinical trial; Received grant/research funds from Hoffmann-La Roche Inc. for clinical trial.

Coauthor(s)

Linda Wagner-Weiner, MD Associate Professor, Department of Pediatrics, University of Chicago, The Pritzker School of Medicine

Linda Wagner-Weiner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Thomas JA Lehman, MD FAAP, FACR, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill Cornell Medical College; Chief, Hospital for Special Surgery

Thomas JA Lehman, MD is a member of the following medical societies: PM American Allergy Society

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

References
  1. Ozen S, Eroglu FK. Pediatric-onset Behçet disease. Curr Opin Rheumatol. 2013 Sep. 25(5):636-42. [Medline].

  2. Talaat RM, Ashour ME, Bassyouni IH, Raouf AA. Polymorphisms of interleukin 6 and interleukin 10 in Egyptian people with Behcet's disease. Immunobiology. 2014 Mar 20. [Medline].

  3. Al-Araji A, Kidd DP. Neuro-Behcet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol. 2009 Feb. 8(2):192-204. [Medline].

  4. Uluduz D, Kürtüncü M, Yapici Z, Seyahi E, Kasapçopur Ö, Özdogan H, et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 2011 Nov 22. 77(21):1900-5. [Medline].

  5. Ebert EC. Gastrointestinal manifestations of Behçet's disease. Dig Dis Sci. 2009 Feb. 54(2):201-7. [Medline].

  6. Al-Dhibi H, Abouammoh M, Al-Harthi E, Al-Gaeed A, Larsson J, Abboud E, et al. Macular hole in Behçet's disease. Indian J Ophthalmol. 2011 Sep-Oct. 59(5):359-62. [Medline]. [Full Text].

  7. Al-Mujaini A, Wali UK. Frosted branch angiitis, neuroretinitis as initial ocular manifestation in Behçet disease. Indian J Ophthalmol. 2011 May-Jun. 59(3):240-1. [Medline]. [Full Text].

  8. Louali FE, Tamdy A, Soufiani A, Oukerraj L, Omari D, Bounjoum F, et al. Cardiac thrombosis as a manifestation of Behçet syndrome. Tex Heart Inst J. 2010. 37(5):568-71. [Medline]. [Full Text].

  9. Seyahi E, Yurdakul S. Behçet's Syndrome and Thrombosis. Mediterr J Hematol Infect Dis. 2011. 3(1):e2011026. [Medline]. [Full Text].

  10. Ilhan B, Can M, Alibaz-Oner F, Yilmaz-Oner S, Polat-Korkmaz O, Ozen G, et al. Fatigue in patients with Behcet's syndrome: relationship with quality of life, depression, anxiety, disability and disease activity. Int J Rheum Dis. 2016 Feb 23. [Medline].

  11. Buyuktas D, Hatemi G, Yuksel-Findikoglu S, Ugurlu S, Yazici H, Yurdakul S. Fatigue is correlated with disease activity but not with the type of organ involvement in Behçet's syndrome: a comparative clinical survey. Clin Exp Rheumatol. 2015 Nov-Dec. 33 (6 Suppl 94):S107-12. [Medline].

  12. Ihle J, Kummerle-Deschner J, Orlikowsky T, Albert E, Niethammer D, Dannecker GE. Factor V Leiden and venous thrombosis in a 4-yr-old girl with Behcet's syndrome. Rheumatology (Oxford). 2000 Feb. 39(2):209-10. [Medline].

  13. [Guideline] Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008 Dec. 67(12):1656-62. [Medline].

  14. Alpsoy E. Behcet's disease: treatment of mucocutaneous lesions. Clin Exp Rheumatol. 2005 Jul-Aug. 23(4):532-9. [Medline].

  15. Fresko I, Soy M, Hamuryudan V, et al. Genetic anticipation in Behcet's syndrome. Ann Rheum Dis. 1998 Jan. 57(1):45-8. [Medline].

  16. Gerber S, Biondi A, Dormont D, Wechsler B, Marsault C. Long-term MR follow-up of cerebral lesions in neuro-Behcet's disease. Neuroradiology. 1996 Nov. 38(8):761-8. [Medline].

  17. Kesen MR, Goldstein DA, Tessler HH. Uveitis associated with pediatric Behcet disease in the american midwest. Am J Ophthalmol. 2008 Dec. 146(6):819-27.e2. [Medline].

  18. Kikuchi H, Aramaki K, Hirohata S. Effect of infliximab in progressive neuro-Behcet's syndrome. J Neurol Sci. 2008 Sep 15. 272(1-2):99-105. [Medline].

  19. Kone-Paut I, Geisler I, Wechsler B, et al. Familial aggregation in Behcet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999 Jul. 135(1):89-93. [Medline].

  20. Marshall SE. Behcet's disease. Best Pract Res Clin Rheumatol. 2004 Jun. 18(3):291-311. [Medline].

  21. Martini A. Behcet's disease and Takayasu's disease in children. Curr Opin Rheumatol. 1995 Sep. 7(5):449-54. [Medline].

  22. Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. J Rheumatol. 2005 Jan. 32(1):98-105. [Medline].

  23. Nakamura S, Ohno S. Anti-tumor necrosis factor alpha antibody in the treatment of Behcet's disease. Int Ophthalmol Clin. 2005. 45(2):179-89. [Medline].

  24. Oktem-Tanor O, Baykan-Kurt B, Gurvit IH, et al. Neuropsychological follow-up of 12 patients with neuro-Behcet disease. J Neurol. 1999 Feb. 246(2):113-9. [Medline].

  25. Rakavor Y, Adar H, Tal I, et al. Behcet Disease: Long-term follow-up of three children and review of the literature. Pediatrics. 1989. 83:986-92. [Medline].

  26. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. 1999 Oct 21. 341(17):1284-91. [Medline].

  27. Tabbara KF, Al-Hemidan AI. Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease. Am J Ophthalmol. 2008 Dec. 146(6):845-50.e1. [Medline].

  28. Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behcet's syndrome. N Engl J Med. 1990 Feb 1. 322(5):281-5. [Medline].

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MRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.
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