How are Bechet's disease symptoms managed?

Management of Behçet's Disease Symptoms

Treatment of Behçet's disease symptoms should be tailored according to the specific organ involvement, with topical treatments for mild mucocutaneous manifestations and immunosuppressive agents for severe or organ-threatening disease. 1

Mucocutaneous Involvement

First-line treatments:

• Topical measures:
  • Topical corticosteroids for oral and genital ulcers 1
  • Lidocaine gel and chlorhexidine for oral ulcers 1
  • Sucralfate suspension for both oral and genital ulcers 1

Systemic treatments:

• Colchicine (1-2 mg/day) should be first-line systemic therapy for:
  • Recurrent mucocutaneous lesions
  • Erythema nodosum (especially effective in women)
  • Genital ulcers 1

For resistant cases:

• Azathioprine
• Thalidomide (caution: teratogenicity and permanent peripheral neuropathy)
• Interferon-alpha
• TNF-alpha inhibitors (infliximab, adalimumab)
• Apremilast 1

Eye Involvement

For posterior segment uveitis:

• Any patient with inflammatory eye disease affecting the posterior segment should be on a treatment regimen including:
  • Azathioprine
  • Systemic corticosteroids (only in combination with immunosuppressives) 1

For severe eye disease (>2 lines drop in visual acuity or retinal involvement):

• Add one of the following to azathioprine and corticosteroids:
  • Cyclosporine-A
  • Infliximab
  • Interferon-alpha 1

Important note:

• Close collaboration with ophthalmologists is essential
• Prompt treatment is critical to prevent vision loss 1

Vascular Involvement

Deep vein thrombosis:

• Immunosuppressive agents:
  • Glucocorticoids
  • Azathioprine
  • Cyclophosphamide
  • Cyclosporine-A 1

Arterial aneurysms:

• High-dose glucocorticoids
• Cyclophosphamide
• Consider TNF-alpha inhibitors in refractory cases 1

Important note:

• Anticoagulants are generally not recommended due to:
  • Risk of bleeding from coexisting pulmonary arterial aneurysms
  • Venous thrombi in Behçet's adhere to vessel walls and rarely cause emboli 1

Neurological Involvement

For parenchymal involvement:

• Acute attacks: High-dose intravenous corticosteroids followed by oral steroids for 6-12 months 2
• Prevention of relapses:
  • Azathioprine
  • Mycophenolate
  • Cyclophosphamide
  • Interferon-alpha
  • TNF-alpha inhibitors 2, 3

For dural sinus thrombosis:

• Corticosteroids
• Consider anticoagulation with immunosuppressants 2, 3

Gastrointestinal Involvement

First-line treatments (before considering surgery except in emergencies):

• Sulfasalazine
• Corticosteroids
• Azathioprine
• TNF-alpha inhibitors
• Thalidomide 1

Joint Involvement

First-line treatment:

• Colchicine (1-2 mg/day) is usually effective 1

For acute monoarticular disease:

• Intra-articular glucocorticoids 1

For recurrent/chronic arthritis:

• Azathioprine
• Interferon-alpha
• TNF-alpha inhibitors 1

Special Considerations

Disease course:

• Behçet's disease typically runs a relapsing and remitting course
• Disease manifestations often ameliorate over time in many patients 1
• Treatment may be tapered and even stopped during the course of the disease

Monitoring:

• Regular follow-up is essential, especially for patients with:
  • Male gender
  • Early age of onset
  • Organ involvement 1

Treatment decisions:

• For skin and mucosa involvement, treatment intensity should match disease severity and impact on quality of life
• For organ-threatening disease (eye, vascular, neurological, gastrointestinal), rapid suppression of inflammation is critical to prevent irreversible damage 1

Remember that Behçet's disease management requires a multidisciplinary approach with specialists experienced in treating this condition to achieve optimal outcomes and prevent long-term morbidity.