What are the treatment options for Bechet's disease?

Treatment Options for Behçet's Disease

Treatment of Behçet's disease should be organ-specific and tailored to disease severity, with immunosuppressive therapy reserved for cases with major organ involvement to prevent irreversible damage and mortality. 1

General Principles

• Behçet's disease is a relapsing-remitting systemic vasculitis that requires prompt suppression of inflammatory exacerbations
• Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis
• Disease manifestations often ameliorate over time, allowing treatment tapering in some cases

Treatment by Organ System

1. Mucocutaneous Involvement

• First-line therapy:
  • Topical steroids for isolated oral and genital ulcers
  • Colchicine (1-2 mg/day) for prevention of recurrent lesions, especially effective for erythema nodosum and genital ulcers in women 1, 2
• For papulopustular or acne-like lesions:
  • Topical treatments as used in acne vulgaris
• For resistant cases:
  • Azathioprine
  • Thalidomide (caution: teratogenicity and peripheral neuropathy)
  • Interferon-alpha
  • TNF-alpha inhibitors
  • Apremilast

2. Eye Involvement

• Posterior segment uveitis requires:
  • Azathioprine as baseline therapy
  • Systemic glucocorticoids (only in combination with immunosuppressives)
  • For severe cases: cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies 1
• For acute sight-threatening uveitis:
  • High-dose glucocorticoids
  • Infliximab or interferon-alpha
  • Intravitreal glucocorticoid injection for unilateral exacerbation

Caution: Cyclosporine-A should not be used in patients with CNS involvement due to potential neurotoxicity 1

3. Vascular Involvement

• For acute deep vein thrombosis:
  • Glucocorticoids and immunosuppressives (azathioprine, cyclophosphamide, cyclosporine-A) 1
  • Anticoagulants are generally not recommended due to risk of bleeding, especially with coexisting pulmonary arterial aneurysms 1
• For arterial aneurysms:
  • Cyclophosphamide and corticosteroids
  • Surgical repair with concurrent immunosuppression for peripheral artery aneurysms

4. Neurological Involvement

• For parenchymal CNS disease:
  • High-dose pulsed corticosteroids (3-7 pulses of IV methylprednisolone 1g/day)
  • Maintenance immunosuppression with azathioprine, cyclophosphamide, methotrexate, TNF-alpha inhibitors, or interferon-alpha 1
• For dural sinus thrombosis:
  • Brief courses of corticosteroids

5. Gastrointestinal Involvement

• Medical therapy should be tried before surgery except in emergencies:
  • Sulfasalazine
  • Corticosteroids
  • Azathioprine (shown to decrease re-operation rates)
  • TNF-alpha antagonists or thalidomide for resistant cases 1

6. Joint Involvement

• First-line therapy:
  • Colchicine 1-2 mg/day (effective in controlling arthritis, especially in men) 1, 2
• For resistant cases:
  • NSAIDs
  • Low-dose corticosteroids
  • Azathioprine

Special Considerations

Leg Ulcers

• May be caused by either venous stasis or obliterative vasculitis
• Treatment approach differs based on cause:
  • Venous stasis ulcers: rest, elevation, topical zinc preparations, good hygiene
  • Vasculitic ulcers: systemic immunosuppressive therapy

Pregnancy

• Colchicine should be avoided due to potential teratogenicity
• TNF-alpha inhibitors may be safer options when treatment is necessary

Disease Monitoring

• Regular ophthalmologic evaluation for patients with eye involvement
• Vascular imaging for patients with vascular manifestations
• Routine laboratory monitoring for patients on immunosuppressive therapy

Treatment Algorithm

1. Assess organ involvement and disease severity
2. For mucocutaneous-only disease: begin with topical treatments and colchicine
3. For major organ involvement (eye, vascular, neurological, GI): initiate appropriate immunosuppressive therapy
4. Adjust treatment based on response and side effects
5. Consider tapering medication if disease remains controlled for extended periods

The treatment of Behçet's disease requires careful monitoring and adjustment based on disease activity and potential medication side effects. Early and appropriate intervention for major organ involvement is crucial to prevent irreversible damage and mortality.