What are the diagnostic criteria and treatment options for Behcet's disease?

Diagnostic Criteria and Treatment Options for Behçet's Disease

Behçet's disease is diagnosed by the presence of recurrent oral ulcers plus at least two of the following: genital ulcers, typical eye lesions (uveitis/retinal vasculitis), characteristic skin lesions, or positive pathergy test, according to the International Study Group criteria. 1

Diagnostic Criteria

Core Diagnostic Features

• Recurrent oral aphthous ulcers: Required as the primary criterion
• Plus at least two of the following:
  • Recurrent genital ulceration
  • Eye lesions: Uveitis (anterior or posterior) or retinal vasculitis
  • Skin lesions: Erythema nodosum, pseudofolliculitis, papulopustular lesions
  • Positive pathergy test (development of papule/pustule 24-48 hours after skin prick)

Additional Diagnostic Considerations

• HLA-B51 association (not diagnostic but supportive) 2
• More common in Mediterranean and Eastern Asian populations 3
• Typically affects young adults between 20-40 years of age 4
• More severe in males with early disease onset 1

Differential Diagnosis

• Sweet's disease
• Pemphigus
• Erythema nodosum (from other causes)
• Crohn's disease 3

Treatment Algorithm Based on Organ Involvement

1. Mucocutaneous Involvement

• First-line treatment:

  • Topical corticosteroids for isolated oral and genital ulcers 1
  • Colchicine (1-2 mg/day) for recurrent mucocutaneous lesions, particularly effective for erythema nodosum and genital ulcers in women 1
  • Topical treatments for acne-like lesions as used in acne vulgaris 1

• For resistant cases:

  • Azathioprine
  • Thalidomide (caution: teratogenicity and peripheral neuropathy)
  • Interferon-alpha
  • TNF-alpha inhibitors 1

2. Eye Involvement

• For posterior segment involvement (sight-threatening):

  • Azathioprine plus systemic corticosteroids as baseline therapy 1
  • For severe eye disease (>2 lines drop in visual acuity or retinal disease):
    • Add either cyclosporine A or infliximab to the baseline therapy
    • Alternatively, interferon-alpha with/without corticosteroids 1
  • Intravitreal glucocorticoid injection for unilateral exacerbations 1

• For isolated anterior uveitis:

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

3. Vascular Involvement

• For deep vein thrombosis:

  • Glucocorticoids and immunosuppressives (azathioprine, cyclophosphamide, or cyclosporine A) 1
  • Anticoagulants are generally NOT recommended due to risk of bleeding from potential coexisting arterial aneurysms 1

• For arterial aneurysms (including pulmonary and peripheral):

  • High-dose glucocorticoids and cyclophosphamide 1
  • Consider TNF-alpha inhibitors for refractory cases
  • For symptomatic patients, surgery or stenting should not be delayed 1
  • Embolization preferred over surgery for patients with high bleeding risk 1

4. Neurological Involvement

• For parenchymal involvement:

  • High-dose pulsed corticosteroids during attacks (3-7 pulses of IV methylprednisolone)
  • Maintenance with oral corticosteroids tapered over 2-3 months
  • Immunosuppressives: Azathioprine, cyclophosphamide, methotrexate 1
  • Avoid cyclosporine A due to potential neurotoxicity 1

• For dural sinus thrombosis:

  • Brief courses of corticosteroids 1

5. Gastrointestinal Involvement

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

Important Clinical Considerations

1. Disease Course: Behçet's disease typically runs a relapsing and remitting course, with manifestations often ameliorating over time 1

2. Poor Prognostic Factors:

   • Ocular involvement (especially posterior segment)
   • Vascular involvement
   • Neurological involvement
   • Gastrointestinal involvement 1
   • Male sex and early age of onset 1

3. Treatment Pitfalls to Avoid:

   • Using anticoagulants without ruling out pulmonary artery aneurysms
   • Using cyclosporine A in patients with neurological involvement
   • Delaying immunosuppressive therapy in sight-threatening ocular disease
   • Relying solely on surgical intervention for gastrointestinal disease without appropriate medical therapy 1

4. Monitoring:

   • Regular ophthalmologic examinations for patients with eye involvement
   • Vascular imaging for patients with vascular manifestations
   • Regular assessment of treatment efficacy and adverse effects

By following this structured approach to diagnosis and treatment based on organ involvement, clinicians can effectively manage Behçet's disease and reduce morbidity and mortality associated with this complex multisystem disorder.