How to assess for a Behcet's disease flare?

Assessment of Behçet's Disease Flare

Assessment of Behçet's disease flare should focus on evaluating the cardinal manifestations including oral ulcers, genital ulcers, skin lesions, and ocular involvement, as well as potential systemic involvement of vascular, neurological, and gastrointestinal systems. 1

Cardinal Manifestations to Assess

Mucocutaneous Manifestations

• Oral ulcers:

  • Evaluate number, size, depth, and pain level
  • Check for oropharyngeal narrowing due to scarring
  • Document healing time and frequency of recurrence

• Genital ulcers:

  • Assess size, depth, and location
  • Look for signs of scarring or deformity
  • Document healing time and frequency of recurrence

• Skin lesions:

  • Erythema nodosum: Check for tender, erythematous nodules typically on lower extremities
  • Papulopustular lesions: Evaluate distribution and extent
  • Pathergy reaction: Test for hypersensitivity to minor trauma (needle prick)
  • Leg ulcers: Differentiate between vasculitic versus venous stasis origin 1

Ocular Involvement

• Assess for:
  • Visual acuity changes (drop of >2 lines indicates severe disease) 1
  • Uveitis (anterior or posterior)
  • Retinal vasculitis
  • Macular involvement
  • Hypopyon (pus in anterior chamber)

Systemic Manifestations

Vascular Involvement

• Evaluate for:
  • Superficial thrombophlebitis
  • Deep vein thrombosis
  • Arterial aneurysms (especially pulmonary and peripheral)
  • Arterial occlusions
  • Vena cava thrombosis or varices 1

Neurological Involvement

• Assess for:
  • Parenchymal CNS lesions (headache, altered mental status, focal deficits)
  • Dural sinus thrombosis (headache, increased intracranial pressure)
  • Meningoencephalitis 1

Gastrointestinal Involvement

• Check for:
  • Abdominal pain
  • Diarrhea
  • Gastrointestinal bleeding
  • Signs of perforation
  • Ulcerations (especially in ileocecal region) 1, 2

Joint Involvement

• Evaluate for:
  • Arthritis (typically non-erosive)
  • Joint pain and swelling
  • Morning stiffness 1

Laboratory Assessment

While no specific diagnostic laboratory test exists for Behçet's disease, the following can help assess disease activity:

• Inflammatory markers:
  • Erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP)
• Complete blood count for anemia or leukocytosis
• HLA-B51 typing (supportive but not diagnostic)

Disease Activity Assessment Tools

The simplified Behçet's Disease Current Activity Form (2006 version) is recommended for standardized assessment of disease activity 3. This helps:

• Quantify disease activity
• Guide treatment decisions
• Monitor response to therapy

Special Considerations

• Severity indicators: Pay particular attention to ocular, vascular, neurological, and gastrointestinal involvement as these may be associated with poor prognosis 1
• Disease course: Note that disease manifestations typically ameliorate over time in many patients 1
• Gender and age considerations: More severe disease course is often seen in men with early age of disease onset 1

Common Pitfalls to Avoid

1. Missing subclinical inflammation: Some patients may have ongoing inflammation without obvious symptoms
2. Attributing symptoms to Behçet's when they're due to other causes: Always consider differential diagnoses like Sweet's disease, pemphigus, erythema nodosum, and Crohn's disease 4
3. Focusing only on mucocutaneous manifestations: Always evaluate for potentially life-threatening systemic involvement
4. Overlooking medication side effects: Some symptoms may be treatment-related rather than disease flares
5. Inconsistent monitoring: Regular follow-up is essential to detect early signs of flare

By systematically evaluating these manifestations, clinicians can accurately assess Behçet's disease flares and make appropriate treatment decisions to prevent irreversible organ damage and improve patient outcomes.