Behcet Syndrome Treatment & Management

  • Author: C Egla Rabinovich, MD, MPH; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Apr 13, 2012
 

Medical Care

  • 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.[8]
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Surgical Care

  • 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

  • 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

  • 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: Abbott Grant/research funds clincal trial; Pfizer Grant/research funds clinical trial; Centacor Grant/research funds clinical trial

Coauthor(s)

Linda Wagner-Weiner, MD  Assistant Professor, Department of Pediatrics, University of Chicago

Linda Wagner-Weiner, MD is a member of the following medical societies: American Academy of Pediatrics and 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 University; Chief, Hospital for Special Surgery

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

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

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, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

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

  3. 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. Sep-Oct 2011;59(5):359-62. [Medline]. [Full Text].

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

  5. 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].

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  7. 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). Feb 2000;39(2):209-10. [Medline].

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

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

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

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  17. [Best Evidence] 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. Jan 2005;32(1):98-105. [Medline].

  18. 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].

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  23. Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behcet's syndrome. N Engl J Med. Feb 1 1990;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.
Histology of ulcers revealing neutrophilic infiltrate and vasculitis.
 
 
 
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