What is the treatment for Behçet’s disease?

Treatment of Behçet's Disease

Treatment for Behçet's disease should be individualized according to the type and severity of organ involvement, with the goal of promptly suppressing inflammatory exacerbations to prevent irreversible organ damage. 1

General Principles

• Behçet's disease typically runs a relapsing and remitting course, with disease manifestations often ameliorating over time 1
• A multidisciplinary approach is necessary for optimal care due to the variety of organ systems that may be affected 1
• Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis and require more aggressive treatment 1

Treatment by Manifestation

Mucocutaneous Involvement

• First-line treatment for oral and genital ulcers: Topical measures such as local corticosteroids 1
• For prevention of recurrent mucocutaneous lesions, especially when erythema nodosum or genital ulcers are dominant:
  • Colchicine should be tried first 1
• For papulopustular or acne-like lesions:
  • Topical or systemic treatments as used in acne vulgaris 1
• For resistant mucocutaneous manifestations, consider:
  • Azathioprine 1, 2
  • Thalidomide (with caution due to teratogenicity and peripheral neuropathy risk) 1
  • Interferon-alpha 1, 2
  • TNF-alpha inhibitors (infliximab, etanercept) 1, 2, 3
  • Apremilast 1

Eye Involvement

• Any patient with Behçet's disease and inflammatory eye disease affecting the posterior segment should be on a treatment regimen including: 1
  • Azathioprine 1
  • Systemic glucocorticoids (only in combination with immunosuppressives) 1
• For severe eye disease (>2 lines drop in visual acuity and/or retinal disease):
  • Cyclosporine-A or infliximab in combination with azathioprine and corticosteroids 1
  • Alternatively, interferon-alpha with or without corticosteroids 1
• For acute sight-threatening uveitis:
  • High-dose glucocorticoids, infliximab, or interferon-alpha 1
  • Intravitreal glucocorticoid injection for unilateral exacerbation as adjunct to systemic treatment 1

Vascular Involvement

• For acute deep vein thrombosis:
  • Immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A 1
• For pulmonary and peripheral arterial aneurysms:
  • Cyclophosphamide and corticosteroids 1
• Note: There is no firm evidence supporting the use of anticoagulants, antiplatelet, or antifibrinolytic agents in the management of vascular manifestations 1

Neurological Involvement

• For parenchymal involvement:
  • Corticosteroids (high-dose pulsed, usually 3-7 pulses of IV methylprednisolone 1 mg/day) 1
  • Followed by maintenance oral corticosteroids tapered over 2-3 months 1
  • Consider: Interferon-alpha, azathioprine, cyclophosphamide, methotrexate, or TNF-alpha antagonists 1
• For dural sinus thrombosis:
  • Corticosteroids 1
• Important: Cyclosporine A should not be used in patients with CNS involvement due to potential neurotoxicity 1

Gastrointestinal Involvement

• Try medical therapy before considering surgery (except in emergencies): 1
  • Sulfasalazine 1
  • Corticosteroids 1
  • Azathioprine 1
  • TNF-alpha antagonists 1
  • Thalidomide 1

Joint Involvement

• In most patients, arthritis can be managed with colchicine 1
• Alternative options include:
  • Indomethacin 1
  • Benzathine penicillin 1

Treatment Approach for Refractory Disease

• For patients with disease resistant to conventional therapies:
  • TNF-alpha inhibitors (infliximab, etanercept, adalimumab) have shown success 3, 4, 5
  • Combination therapy (e.g., infliximab plus methotrexate) may be effective for recalcitrant cases 5
  • Interferon-alpha has shown promising results in open studies 3

Important Considerations

• Treatment should be more aggressive in men with early age of disease onset due to typically more severe disease course 1
• As disease manifestations usually abate over time, treatment may be tapered and even stopped during the course of the disease 1
• The ultimate goal of treatment is to induce and maintain remission, preventing irreversible organ damage 1