What is the treatment for Behcet's disease?

Treatment of Behçet's Disease

Treatment of Behçet's disease should be organ-specific and tailored to disease severity, with colchicine as first-line therapy for mucocutaneous manifestations and immunosuppressives for major organ involvement. 1

Treatment Algorithm by Organ System

Mucocutaneous Manifestations

• First-line therapy:
  • Topical steroids for oral and genital ulcers 1
  • Colchicine (1-2 mg/day) for prevention of recurrent lesions, particularly effective for erythema nodosum and genital ulcers 1, 2
• Second-line therapy for refractory cases:
  • Azathioprine
  • Thalidomide
  • Interferon-alpha
  • TNF-alpha inhibitors
  • Apremilast 3
• For papulopustular/acne-like lesions:
  • Treat as in acne vulgaris with topical or systemic measures 1

Ocular Involvement

• Posterior segment disease (sight-threatening):
  • Requires immunosuppressive therapy 1
  • Azathioprine with systemic glucocorticoids as baseline therapy 1
  • For severe eye disease (>2 lines drop in visual acuity and/or retinal disease):
    • Add cyclosporine-A or infliximab to azathioprine and corticosteroids 1
    • Alternatively, interferon-alpha with or without corticosteroids 1
  • Acute sight-threatening episodes: high-dose glucocorticoids, infliximab, or interferon-alpha 1
  • Intravitreal glucocorticoid injection for unilateral exacerbation as adjunct to systemic treatment 1

Isolated Anterior Uveitis

• Consider systemic immunosuppressives for poor prognostic factors (young age, male sex, early disease onset) 1

Vascular Involvement

• Deep vein thrombosis:
  • Glucocorticoids and immunosuppressives (azathioprine, cyclophosphamide, or cyclosporine-A) 1
  • Monoclonal anti-TNF antibodies for refractory cases 1
  • Important: Anticoagulants are not routinely recommended due to risk of bleeding from potential coexisting arterial aneurysms and lack of evidence for benefit 1
• Arterial aneurysms:
  • Cyclophosphamide and corticosteroids 1

Neurological Involvement

• Parenchymal involvement:
  • Corticosteroids, interferon-alpha, azathioprine, cyclophosphamide, methotrexate, or TNF-alpha antagonists 1
• Dural sinus thrombosis:
  • Corticosteroids 1

Gastrointestinal Involvement

• Medical therapy (try before surgery except in emergencies):
  • Sulfasalazine
  • Corticosteroids
  • Azathioprine
  • TNF-alpha antagonists
  • Thalidomide 1

Joint Involvement

• Colchicine (1-2 mg/day) is usually effective 1
• Arthritis in Behçet's typically follows a mild, non-erosive course affecting large joints 1

Treatment Principles and Caveats

1. Disease course considerations:

   • Disease activity often decreases over time, allowing for tapering and potentially stopping treatment 1, 2
   • More aggressive treatment is needed in early disease, especially in young males 4

2. Treatment goals:

   • Promptly suppress inflammatory exacerbations
   • Prevent irreversible organ damage 1, 2

3. Common pitfalls to avoid:

   • Anticoagulation: Avoid routine use in venous thrombosis as thrombi adhere to vessel walls and rarely embolize; risk of fatal bleeding if pulmonary arterial aneurysms coexist 1
   • Delayed treatment: Early diagnosis and prompt treatment are crucial to prevent irreversible damage 2
   • Inappropriate monotherapy: Severe ocular, vascular, or neurological disease often requires combination therapy 1

4. Monitoring:

   • Regular assessment for disease activity and treatment-related complications
   • Multidisciplinary approach involving rheumatology, ophthalmology, dermatology, and other specialties as needed 2

By following this organ-specific approach and recognizing the relapsing-remitting nature of Behçet's disease, treatment can effectively reduce morbidity and mortality associated with this complex multisystem disorder.