Adamantiades-Behçet disease is a multi-system inflammatory disease of unknown etiology, classified as systemic vasculitis involving all types and sizes of blood vessels and characterized clinically by recurrent oral aphthous and genital ulcers, skin lesions, and iridocyclitis/posterior uveitis, occasionally accompanied by arthritis and vascular, gastrointestinal, neurologic, or other manifestations.
- Specific challenges associated with the recognition of the condition
Delayed diagnosis (7 years). International clinical diagnostic criteria published by the group. No biomarker available.
- Specific challenges associated with the diagnosis
Disease not expected in patients of caucasian origin. Sex- and age-associated variability of the clinical picture.
- Specific challenges associated with care of these patients over their lifespan – Quality of life issues – Gaps accross the care
Chronic, recurrent disease. A severe course (blindness, meningoencephalitis, hemoptysis, intestinal perforation, severe arthritis) occurs in approx. 10%. Blindness may be prevented with early aggressive therapy of posterior uveitis. Lethal outcome in 0-6% of patients. Central nervous system, pulmonary and large vessel involvement, bowel perforation are major life-threatening complications. Markers of severe prognosis: HLA-B51 positivity, male gender, early systemic signs.
Incidence : 0.75-1 / 100 000 inhabitants per year
Prevalence : 0.27-1.18 / 100 000 (northern Europe), 1.5-7.5/100 000 (southern Europe)