How is Behçet disease treated?

Updated: Dec 21, 2020
  • Author: Nicole Davey-Ranasinghe, MD; Chief Editor: Herbert S Diamond, MD  more...
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For oral and genital ulcerations, topical steroids or sucralfate solution are first-line therapy for mild isolated ulcerations. Colchicine has also been used to prevent mucocutaneous relapse. [47, 48] For severe mucocutaneous lesions, systemic corticosteroids, azathioprine, pentoxifylline, dapsone, interferon-alfa, colchicine, and thalidomide have demonstrated benefit.

For ocular disease, azathioprine is widely accepted as the initial agent. For severe eye disease (significant drop in visual acuity, retinal vasculitis, or macular involvement), either cyclosporine or infliximab may be used in combination with azathioprine and corticosteroids. [49, 50, 45, 51] An expert panel has recommended considering infliximab and adalimumab as first-line immunomodulatory agents for the treatment of ocular manifestations. [52]

Interferon-alfa, alone or in combination with corticosteroids, appears to be a second choice in severe ocular Behçet disease. [53, 54] It is used primarily in Europe. A retrospective study of a large case series from Germany reported long-lasting remission and improved visual prognosis in patients with severe ocular Behçet disease treated with alpha interferon. [53]

For gastrointestinal lesions, treatment alternatives based on expert opinion are aminosalicylate (5-ASA) derivatives, including sulfasalazine or mesalamine; systemic corticosteroids, azathioprine, tumor necrosis factor–α (TNF-α) antagonists, and thalidomide.

Arthritis may respond to prednisone, local corticosteroid injections, nonsteroidal anti-inflammatory drugs (NSAIDs), and colchicine. Interferon-alfa, azathioprine, and TNF-α blockers may be tried in rare cases of patients with resistant, prolonged, and disabling attacks.

Cutaneous disease with erythema nodosum is a special circumstance and may be treated with colchicine or dapsone.

Central nervous system (CNS) disease is usually treated with systemic corticosteroids, interferon-alfa, azathioprine, cyclophosphamide, [55] methotrexate, and TNF-α antagonists. [56]

Major-vessel disease with thrombotic events are treated with systemic anticoagulation in addition to corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A. Pulmonary arterial aneurysms are treated with cyclophosphamide and corticosteroids.

TNF-α antagonists are increasingly used and have become standard treatment of Behçet disease that is inadequately controlled by standard immunosuppressive regimens. [57, 58] Infliximab has been most widely studied, but adalimumab has proved successful in cases refractory to both conventional therapy and infliximab. [59] Etanercept is the only TNF inhibitor with data from a short-term randomized controlled study demonstrating efficacy in suppressing most of the mucocutaneous manifestations of Behçet disease.  [60]

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