Behcet’s Disease

Table of contents
Key points ⚡
Succinct notes to superpower your revision
Behcet’s disease\: autoimmune vasculitis characterised by oral and genital ulcers and uveitis; most common in
Mediterranean, Middle Eastern, and East Asian populations.
Aetiology\: unknown; potential genetic basis linked to HLA-B51/B5; non-Mendelian inheritance; possible trigger by
S t a p h y l o c o c c u s a u r e u s or other antigens.
Risk factors\: Mediterranean, Middle Eastern, and East Asian ethnicity; age 20-30 years; HLA-B51 carrier; males tend to have
more severe presentations.
Clinical features\: classic triad of oral ulcers, genital ulcers, and uveitis; other manifestations include memory impairment,
loss of coordination, DVT, gastrointestinal ulceration, arthritis, fever, and rash (erythema nodosum).
Di
arteritis, sarcoidosis, squamous cell carcinoma.
Investigations\: pathergy test (positive if papule forms at pinprick site); FBC (anaemia, leukocytosis), elevated ESR/CRP,
antiphospholipid antibodies, HLA-B51 testing; imaging (X-rays, CT, MRI) as indicated.
Diagnosis\: clinical, based on The International Clinical Criteria for Behcet’s Disease Classi
two of genital ulcers, uveitis, skin lesions, or positive pathergy test.
Management\: corticosteroids and colchicine for oral and genital ulcers; immunosuppressants (e.g., azathioprine, anti-TNF-a
antibodies) if needed; corticosteroids and immunosuppressants for systemic involvement; ophthalmology referral for eye
symptoms.
Complications\: increased risk of visual impairment, blindness, CNS defects, cardiovascular complications, gastrointestinal
haemorrhage, thrombosis; risk of coronary and pulmonary aneurysms.
Prognosis\: variable; worse in men and younger patients; complications like ruptured aneurysms, renal amyloidosis, and
neurological sequelae worsen prognosis; proper management allows most to live normal lives.
Article 🔍
A comprehensive topic overview

Introduction

Behcet’s disease is presumed to be an autoimmune vasculitis, though its cause is unknown. It is characterised by oral and
genital ulcer formation with uveitis.
Behcet’s disease is most prevalent in Mediterranean, Middle Eastern, and East Asian populations. Turkey has the highest
prevalence at 420 cases per 100,000 population. In the United States, the prevalence is much lower at roughly 5 per
100000 population.
1,2

Aetiology

The aetiology of Behcet’s disease is currently unknown.
Epidemiological studies suggest that there may be a genetic basis to the development of Behcet’s disease, as those with
HLA-B51/B5 carriers are most likely to develop the condition. However, the inheritance of Behcet’s disease is non-
Mendelian.
2One current theory is that exposure to S t a p h y l o c o c c u s a u r e u s or to other pathogenic antigens may serve as a trigger for
the development of the autoimmune response.
2

Risk factors

Risk factors for Behcet’s disease include\:
1,2
Mediterranean, Middle Eastern, and East Asian ethnicity
Age 20–30 years
HLA-B51 carrier
Disease prevalence is roughly equal among males and females but males tend to have more severe presentations.

Clinical features

The classic triad of the disease is oral ulcers, genital ulcers, and uveitis.
Multiple painful ulcers of the oral mucosa are the hallmark of Behcet’s disease. These can present anywhere in the oral
mucosa and are recurrent, often last for several weeks before healing.
1
Genital ulcers often occur on the penis in males and are extremely painful. They may also last several weeks before
resolving. However, unlike oral ulcers, these are more likely to scar.
2
Uveitis may develop several years after the initial presentation of the disease. Patients may complain of bilateral vision
loss.
2
Other systemic manifestations include\:
1,2
Memory impairment
Loss of coordination
Deep vein thrombosis
Gastrointestinal ulceration
In
Fever
Rash
History
Typical symptoms of Behcet’s disease include\:
Painful ulcers in the mouth (Figure 1)
Painful ulcers of the genitalia
Blurred vision
Joint pains
Fever
Figure 1. Oral ulcers associated with
Behcet’s disease.
3
Clinical examination
Typical clinical
2
Ulcers of the mouth and genitalia
Reduced visual acuityErythema of the eyes (Figure 2)
Erythema nodosum
Figure 2. Uveitis and hypopyon in a
patient with Behcet’s disease.
4

Di

Several other diseases can mimic both the ulcers and the other systemic features seen in Behcet’s disease\:
1,2
Crohn’s disease
Herpes simplex virus
Hand foot and mouth disease
Systemic lupus erythematosus
Seronegative arthritis
Multiple sclerosis
Takasyu’s arteritis
Sarcoidosis
Squamous cell carcinoma

Investigations

Most investigations in the work-up of Behcet’s disease are performed for the purposes of ruling out other diagnoses.
Bedside investigations
Relevant bedside investigations include\:
Pathergy testing\: though this is less commonly positive in Caucasian patients.
5
Pathergy test
The pathergy test is a hypersensitivity reaction to pinprick. A small needle is inserted into the skin and the needle
site is evaluated at 24 and 48 hours.
A positive
5
Laboratory investigations
Relevant laboratory investigations include\:
1
Haematology\: full blood count (may show anaemia of chronic disease and leukocytosis)
Biochemistry\: elevated in
Immunology\: antiphospholipid antibodies (may be positive in some cases)
Genetics\: HLA-B51 testing
Imaging
Relevant imaging investigations depend on clinical indications but may include\:
1
X-rays of a
CT angiography\: to evaluate for the presence of large vessel thrombosis or aneurysm
MRI brain

Diagnosis

The diagnosis of Behcet’s disease is entirely clinical, and there are no de
diagnosis.
The diagnostic criteria for Behcet’s disease are described in The International Clinical Criteria for Behcet’s Disease
Classi
Any two of the following\:
6
Recurrent genital ulcerations
Uveitis or retinal vasculitis
Skin lesions
Positive pathergy test

Management

The management of Behcet’s disease is aimed at controlling symptoms and in
7,8
Corticosteroids and colchicine are used as
Immunosuppressive agents (e.g. azathioprine, anti-tumour necrosis factor-a antibodies) should be used in select cases
when the previously mentioned drugs are unsuccessful.
For patients with additional systemic involvement, corticosteroids and immunosuppressant medications are used to reduce
in

Complications

Behcet’s disease with eye involvement increases the risk of permanent visual impairment and blindness.
Systemic involvement can increase the risk of central nervous system defects, cardiovascular complications,
gastrointestinal haemorrhage, and thrombosis.
2
Vascular in
mortality.
2
Prognosis
Prognosis is variable and can depend on many factors.
In some patients, systemic complications can lead to early mortality. Complications associated with a worse prognosis
include ruptured aneurysms, renal amyloidosis, and neurological sequelae. Prognosis is worse in men and those diagnosed
at a younger age.
2
With proper medical management, most patients can live normal lives.
6

References

Tidy C, Cox J. B e h c e t’ s D i s e a s e . Reviewed in 2016. Available from\: [LINK]
Adil A, Goyal A, Bansal P, Quint P. B e h c e t D i s e a s e . StatPearls. Updated in 2021. Available from\: [LINK]
Altiner A, Mandal R. O r a l u l c e r i n p a t i e n t w i t h B e h c e t’ s d i s e a s e . Licence\: [CC BY-SA 3.0]
EyeMD. A n t e r i o r u v e i t i s a n d h y p o p y o n i n a p a t i e n t w i t h B e h c e t’ s d i s e a s e . Licence\: [CC BY-SA 2.5]Shaoon R, Daveluy S. P a t h e r g y T e s t . StatPearls. Updated in 2021. Available from\: [LINK]
American Behcet’s Disease Association. D i a g n o s i s o f B e h c e t’ s D i s e a s e . Published in 2018. Available from\: [LINK]
Haterni G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2 0 1 8 U p d a t e o f t h e E U L A R r e c o m m e n d a t i o n s f o r t h e
m a n a g e m e n t o f B e h c e t’ s s y n d r o m e . Annals of the Rheumatic Diseases 2018;77\:808-818. Available from\: [LINK]
National Health Service. T r e a t m e n t \: B e h c e t’ s d i s e a s e . Reviewed in 2019. Available from\: [LINK]

Reviewer

Dr Grainne Murphy
Consultant Rheumatologist
Cork University Hospital

Related notes

Juvenile Idiopathic Arthritis
Osteoporosis
Polymyalgia Rheumatica
Psoriatic Arthritis
Rheumatic Fever

Test yourself

Contents

Introduction
Aetiology
Risk factors
Clinical features
Di
Investigations
Diagnosis
Source\: geekymedics.com