Printed on: March 6th, 2025
Behçet’s syndrome
Behçet’s syndrome
Behçet’s syndrome, also known as Behçet’s disease, is a rare multi-system inflammatory condition. In the UK, the most common presentation is with skin inflammation and mucous membrane ulceration, but the condition has significant geographic variation both in incidence and in the type of presentation. It is a complex disease, and management will be led by teams in secondary care. In primary care, we can help by being aware of when to suspect and refer, and by having some understanding of the associated risks to watch out for.
This article uses information from a 2024 British Association of Dermatology (BAD) and British Society of Rheumatology (BSR) joint guideline, and also from reviews in the Lancet and the New England Journal of Medicine (BJD 2024;191:e8, Lancet 2024;403:1093, NEJM 2024;340:640).
This article was updated in December 2024.
Incidence and epidemiology
Peak incidence of Behçet’s is between 20 and 40 years of age, and presentation in childhood or past the age of 50 is rare (Lancet 2024;403:1093). If children do develop Behçet’s, the presenting symptoms are often musculoskeletal or gastrointestinal rather than mucocutaneous, making diagnosis a challenge.
It is more common in men than women.
Worldwide prevalence is estimated at around 10/100 000 people (Lancet 2024;403:1093), but there is high geographic variability. There are around 1000 people in the UK living with the condition (BAD Patient Hub - Behçets).
Pathogenesis
The pathogenesis of Behçet’s is not fully understood, but seems to be multi-factorial with a genetic predisposition, followed by exposure to environmental triggers such as infection, gut and oral microbiome imbalance, stress, poor oral hygiene and a diet high in histamine-containing foods (NEJM 2024;340:640). It shows features of both autoimmune and auto-inflammatory pathology.
Diagnosis
Diagnosis is clinical, with no universally-accepted diagnostic criteria and no validated biomarker we can use for confirmation (Lancet 2024;403:1093).
The BAD/BSR guideline recommends using one of 2 diagnostic tools: the International Study Group 1990 tool or the International Criteria for Behçet’s disease, updated in 2024.
| International Study Group Criteria 1990 | International Criteria for Behçet’s 2024 |
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The pathergy test: a non-specific skin hyperreactivity response is seen after an intradermal skin prick (the guidelines do not discuss using this test in primary care).
| Presentation by body system (NEJM 2024;340:640 unless otherwise indicated) |
| Brain (affects 10–30% of people): |
| Eyes (affects 50% of people): |
| Skin and mucosa: |
| Vascular (affects up to 40% of people): |
| GI tract (40–60% of people in the UK will have GI involvement (Lancet 2024;403:1093). Worldwide rates are generally lower (NEJM 2024;390:640)): |
| Musculoskeletal (affects 50% of people): |
Management
If we suspect Behçet’s, we should refer to secondary care for MDT review and diagnostic assessment (BJD 2024;191:e8). The summary below is taken from the BAD/BSR joint guideline, which goes into detail on the secondary care management of the different disease manifestations.
In secondary care
At MDT review, the specialist teams should use a disease-specific tool to assess disease activity and quality of life.
Treatment depends on the manifestation of the disease, but will usually involve potent topical corticosteroids followed by systemic steroids (oral or IM), with disease-modifying steroid-sparing drugs once the condition is stabilised. Combinations of DMARDs, anti-TNF drugs and biologics are often used. Musculoskeletal symptoms may respond to colchicine.
Prognosis
Behçet’s shows a variable course, with a relapsing and remitting pattern (NEJM 2024;390:640). Prognosis is worse in those diagnosed at a younger age and in those with higher degree of disease activity. Males are 5x more likely to die of the condition. Vascular disease carries the highest mortality. Overall mortality is estimated at almost 10% over the 20 years after diagnosis.
Regardless of which body systems are impacted, there is a significant adverse effect on quality of life, affecting both physical and psychological wellbeing.
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Behçet’s syndrome |
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