What are the treatment options for Bechet's disease?

Treatment Options for Behçet's Disease

Treatment for Behçet's disease should be organ-specific and tailored to the type and severity of manifestations, with immunosuppressive therapy required for potentially life-threatening or organ-damaging involvement. 1

Overarching Treatment Principles

• Behçet's disease typically runs a relapsing and remitting course
• Goal of treatment: promptly suppress inflammatory exacerbations to prevent irreversible organ damage
• Disease severity varies by organ involvement (eye, vascular, neurological, and gastrointestinal involvement carry worse prognosis)
• Disease manifestations often ameliorate over time in many patients

Treatment by Organ System Involvement

1. Mucocutaneous Involvement

• First-line for oral/genital ulcers: Topical steroids 1
• First-line for prevention of recurrent lesions: Colchicine (1-2 mg/day) 1, 2
  • Particularly effective for erythema nodosum and genital ulcers in women 2
  • Shown to reduce arthritis in both men and women 2
• For papulopustular/acne-like lesions: Topical treatments as used in acne vulgaris 1
• For resistant cases: Consider azathioprine, thalidomide, interferon-alpha, TNF-alpha inhibitors, or apremilast 1

2. Eye Involvement

• Posterior segment uveitis: Requires aggressive immunosuppression 1

  • Base treatment: Azathioprine plus systemic corticosteroids 1
  • For severe disease (>2 lines drop in visual acuity or retinal vasculitis): Add either cyclosporine A or infliximab; alternatively, interferon-alpha with/without corticosteroids 1
  • Important: Systemic corticosteroids should only be used in combination with immunosuppressives, never as monotherapy 1

• Acute sight-threatening uveitis: High-dose glucocorticoids, infliximab, or interferon-alpha 1

  • Consider intravitreal glucocorticoid injection for unilateral exacerbation 1

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

3. Vascular Involvement

• Acute deep vein thrombosis: Glucocorticoids plus immunosuppressives (azathioprine, cyclophosphamide, or cyclosporine-A) 1

  • Important: Anticoagulants are not routinely recommended due to risk of aneurysm rupture 1
  • Venous thrombi in Behçet's adhere to vessel walls and rarely cause emboli 1

• Arterial aneurysms: Cyclophosphamide plus corticosteroids 1

  • Surgical repair may be necessary with concurrent immunosuppression 1

4. Neurological Involvement

• Parenchymal CNS disease: Corticosteroids, interferon-alpha, azathioprine, cyclophosphamide, methotrexate, or TNF-alpha antagonists 1

  • High-dose pulsed corticosteroids (3-7 pulses of IV methylprednisolone 1g/day) during attacks 1
  • Maintenance oral corticosteroids tapered over 2-3 months 1

• Dural sinus thrombosis: Brief courses of corticosteroids 1

• Important: Cyclosporine A should be avoided in patients with neurological involvement due to potential neurotoxicity 1

5. Gastrointestinal Involvement

• First-line: Sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide 1
  • Medical treatment should be tried before surgery except in emergencies 1
  • Azathioprine may decrease re-operation rates in patients requiring surgery 1

6. Joint Involvement

• First-line: Colchicine 1-2 mg/day 1, 2
  • Effective for both men and women with arthritis 2
  • Arthritis in Behçet's typically follows mild, transient course without deformities or erosions 1

Special Considerations

• Gender differences: Disease tends to be more severe in men, especially with early age of onset 1, 3
• Colchicine efficacy: More effective in women for genital ulcers and erythema nodosum 2
• Treatment tapering: As disease manifestations typically abate over time, treatment may be tapered and even stopped during the course of disease 1
• Monitoring: Regular follow-up is essential, particularly for patients with high-risk organ involvement

Common Pitfalls to Avoid

1. Using cyclosporine A in patients with neurological involvement
2. Using anticoagulants in vascular Behçet's without careful consideration of bleeding risk
3. Using corticosteroids as monotherapy for ocular disease
4. Delaying immunosuppressive therapy in sight-threatening eye disease
5. Rushing to surgery for gastrointestinal involvement before adequate trial of medical therapy

The treatment approach should be guided by the specific organ involvement, with more aggressive therapy reserved for manifestations that threaten mortality or cause permanent damage (eye, vascular, neurological, and gastrointestinal).