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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

See the list below:

  • 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.

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.


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.


See the list below:

  • 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.


See the list below:

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

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.


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.

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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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