Behçet’s

Syndrome
Behçet's syndrome, also known as Behçet's disease,
is a rare and chronic inflammatory disorder that
affects blood vessels throughout the body.

An autoinflammatory condition is where there are

repeated episodes of inflammation in the body.

Behçet's disease is considered an

autoimmune disease

Hulusi Behçet
Behçet's disease
The exact cause of Behçet's syndrome is not well understood, and it is considered a complex
disorder with a multifactorial origin

1. Genetic Factors: Certain genetic factors,

including specific human leukocyte antigen
(HLA) types such as HLA-B51, have been found to
be more prevalent in individuals with Behçet's
syndrome.

2. Environmental factors : may trigger the onset or

exacerbation of Behçet's syndrome.
Environmental triggers could include infections,
although no specific infectious agent has been
consistently linked to the syndrome.
The exact cause of Behçet's syndrome is not well understood, and it is considered a complex
disorder with a multifactorial origin

3. Imbalance in Immune Regulation: There may

be a dysfunction in the regulation of the immune
system in individuals with Behçet's syndrome. This
dysregulation could result in chronic inflammation
and the formation of characteristic lesions and
ulcers.
 Environmental trigger
Genetic predisposition

Anabell, a 30-year-old woman of Middle Eastern

Anabell encounters a viral infection, let's
descent, inherits specific human leukocyte
antigen (HLA) genes, including HLA-B51, from her call it "Virus X," in her environment.
parents
Immune System Activation
Anabell's, immune system responds to the viral infection by
activating immune cells, particularly T cells.

The presence of HLA-B51 may

Antigen
influence how T cells respond to
Presentation the viral antigens.

HLA-B51
HLA-B51 + failure in immune tolerance
immune system fails to distinguish
between self and non-self
viral infection

Annabell’s immune T cells, instead of recognizing the

system mistakenly T cells initiate an presented antigens as harmless,
targets her own cells, autoimmune response start attacking Annabell’s own
considering them as tissues.
foreign invaders.

infiltration of T cells Chronic

v Inflammation Inflammation
into blood vessels

VASCULAR INFLAMMATION
Continues releasing of
pro-inflammatory
cytokines by T cells
and other immune
cells
 SIGNS AND SYMPTOMS The inflammatory
Annabell experiences
symptoms such as
process extends redness, pain, and
VASCULAR INFLAMMATION to the eyes, blurred vision due to
leading to inflammation in the
particularly in mucous
membranes, causing
UVEITIS uvea.

recurrent oral and

can lead to the formation of
genital ulcers.
ulcers in different parts of the
body
 SIGNS AND SYMPTOMS
Skin Lesions and Joint
Involvement
Inflammation may manifest as
skin lesions, including red,
raised nodules.
Annabell also develops
arthritis, experiencing joint
pain and swelling.
Neurological Complications
In some cases, Behçet's
disease can involve the
nervous system.
Annabell may experience
neurological complications,
such as:
• inflammation of the brain
or spinal cord
Meningitis
Vascular Complications
Severe cases of Behçet's
disease can involve vascular
complications, including:
• blood clots and
aneurysms, due to the
ongoing damage to blood
vessels
Diagnostic
Tests
1. HLA-B51 Genetic Marker
2. Skin Pathology
3. Oral and Genital Ulcers
4. Eye Examination
5. Blood Tests
1. HLA-B51 Genetic Marker
- the presence of this genetic marker is
not exclusive to the disease, and many
individuals with Behçet's do not have the
HLA-B51 marker.

2. Skin Pathology
- skin biopsy may be performed to
examine the characteristic features of
the lesions, such as vasculitis
(inflammation of blood vessels).
3. Oral and Genital Ulcers
- Behçet's syndrome often involves
recurrent oral and genital
ulcers. The presence and
characteristics of these ulcers
can aid in the diagnosis.
4. Eye Examination
-Ocular involvement is common in
Behçet's syndrome, and it can lead
to serious complications
- eye examination is done to check for
uveitis, retinal vasculitis, or other
eye-related manifestations.

5. Blood Tests
- various blood tests may be performed
to rule out other conditions and
assess inflammation. This may
include erythrocyte sedimentation
rate (ESR) and C-reactive protein
(CRP) levels.
 MEDICAL MANAGEMENT
1. Nonsteroidal Anti-Inflammatory Drugs
(NSAIDs)
- ibuprofen or naproxen may be used to
manage pain and inflammation. However, they
do not alter the course of the disease itself.
2. Corticosteroids
- prednisone may be prescribed to control
inflammation during flare-ups
- often used in higher doses during acute
episodes and then tapered down to lower
maintenance doses.
3. Immunosuppressive Medications
- cases where symptoms are severe or
involve internal organs, immunosuppressive
drugs may be prescribed to modulate the
immune response
- azathioprine, methotrexate, cyclosporine,
and mycophenolate mofetil.

4. Colchicine
- reduce the frequency and severity of oral and genital
ulcers in Behçet's syndrome.
6. Thalidomide and Lenalidomide
- may be considered for severe cases of Behçet's syndrome,
particularly when there is ocular involvement.

7. Topical Treatments
- For skin manifestations

8. Ocular Treatments
- Corticosteroid eye drops, immunosuppressive medications, and
sometimes surgical interventions may be necessary to manage
ocular manifestations.
 Nursing Management
1. Patient Education
- Educate the patient about the importance of adherence to prescribed
medications and the potential side effects.
- Discuss the need for regular follow-up appointments with healthcare
providers for monitoring and adjustments to the treatment plan

2. Pain Management
- Assess and monitor the patient's pain levels regularly, especially during
flare-ups.
- Collaborate with the healthcare team to develop and implement
effective pain management strategies.
- Administer prescribed pain medications as directed.
3. Skin Care
- Encourage the patient to avoid trauma to the skin,
such as excessive scratching or picking. Monitor
and document the appearance and progression of
skin lesions.

4. Oral Hygiene
- prevent and manage oral ulcers. Recommend
mild, non-irritating oral care products. Suggest
avoiding spicy or acidic foods that may exacerbate
oral ulcers.

5. Genital Care: Provide guidance on managing genital

ulcers, including the use of prescribed topical treatments.
Encourage the use of loose-fitting clothing to minimize
irritation. Address any concerns or questions related to sexual
health.
6. Emotional Support: Assess the patient's emotional well-
being and provide a supportive environment. Refer patients
to counseling or support groups to cope with the emotional
impact of chronic illness. Collaborate with mental health
professionals when needed.

7. Adherence to Medications: Assess the patient's

understanding of prescribed medications and potential side
effects. Develop strategies to promote medication
adherence, such as creating a medication schedule or using
pill organizers. Monitor for medication side effects and report
any concerns to the healthcare team.
8. Ocular Care: Encourage regular eye examinations by an
ophthalmologist. Educate the patient about symptoms of ocular
involvement and the importance of prompt reporting to healthcare
providers.

9. Assistance with Activities of Daily Living: Assess the patient's

functional status and provide assistance with activities of daily living
as needed during periods of increased symptoms. Collaborate with
occupational therapists to address specific challenges related to
daily activities.