Behcet Syndrome Treatment & Management

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

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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