Behcet's Disease

What is Behcet’s Disease?

Behcet's disease, also known as Behcet's syndrome or Silk Road disease, is a rare genetic condition characterized by inflammation of blood vessels (vasculitis). This condition affects the arteries and veins, resulting in a variety of symptoms. It is most commonly seen in people aged 20 to 30 and is found in Turkey, the Middle East, and Asia.

Causes of Behcet’s Disease

Behcet’s disease is an autoimmune disorder, the exact cause of which is still unknown.

Studies suggest that a gene may be present in the body but will be expressed once it is triggered by environmental factors. The body’s immune system mistakenly attacks the immune cells resulting in an inflammatory response all over the body.

Symptoms of Behcet’s Disease

Symptoms of Behcet’s disease may vary among individuals and include:

• Sores in the mouth and genitals: Painful sores similar to canker sores arise inside the cheeks, tongue, lips, and scrotum in men and genitalia in women.
• Inflammation in the eye: This is the most serious symptom resulting in:
  • Uveitis (swelling in the front or back of the eye)
  • Vision loss
  • Redness
  • Pain
  • Sensitivity to light
• Skin problems: These are common symptoms appearing as acne, boils, ulcers, red patches, and spots like insect bites.
• Joint pain: Inflammation, pain, swelling, tenderness, and redness in the elbows, ankles, and other joints may be noted.
• Vasculitis: Inflammation in the vein can lead to the formation of clots and blockages resulting in pain, and redness in the arms and legs. Inflammation in large arteries can lead to an aneurysm.
• Inflammation of the Brain and Nervous System: This is one of the more serious symptoms that could result in a stroke, headache, confusion, personality changes, and in rare cases, dementia.
• Ulcers in Digestive System: Ulcers may be formed in the gastrointestinal tract anywhere from the mouth to the anus. The symptoms include bloody diarrhea, abdominal pain, and inflammation of the bowel.

Diagnosis of Behcet’s Disease

Behcet’s disease does not contain any specific diagnostic test to confirm the condition as the symptoms are common for other conditions. Your doctor will make a clinical diagnosis based on symptoms and certain diagnostic tests, which include:

• Pathergy Test: This is a pin-prick test where multiple pricks are made into the forearm using a sterile needle and the area is examined after two days. If small red bumps appear on the pricked area, the test is considered positive.
• Blood test: Blood tests are performed to look for certain substances or the presence of elevated enzymes in the blood that can indicate infection or disease. The various tests include:
  • Erythrocyte Sedimentation Rate (ESR)
  • C-reactive Protein (CRP)
  • Total Blood Count
  • Kidney Function Test
  • Liver Function Test
• Chest X-rays: During this study, high electromagnetic energy beams produce images of the chest to determine the presence of infection in the lungs.
• Endoscopy: This is a diagnostic procedure that involves the use of a long, thin, flexible tube with a tiny video camera and light on one end to produce images of the GI tract.
• Magnetic Resonance Imaging (MRI) Scan: An imaging study that uses a large magnetic field and radio waves to detect any damage to the tissues.
• Computed Tomography (CT) Scan: This scan uses multiple x-rays to produce detailed cross-sectional images of the body.
• Lumbar puncture: Also known as a spinal tap, this is a procedure in which fluid is aspirated from the spinal canal and analyzed to detect inflammation or the presence of any antibodies to rule out any infections.

Treatment for Behcet’s Disease

Treatment for Behcet’s syndrome may vary based on the symptoms and severity of the condition and only helps to relieve the symptoms. The treatment method includes:

• Topical Therapy: This is a pain-relieving corticosteroid that is applied to the skin and genital sores to reduce inflammation and pain. The medication can be applied in the form of gels, eye drops, and ointments.
• Immunosuppressants: Immunosuppressant drugs such as azathioprine cyclosporine and cyclophosphamide prevent the immune system from attacking healthy tissues.
• Biological therapies: Drugs such as infliximab and adalimumab target the biological processes that regulate the activity of the immune system to control inflammation.
• Lifestyle Modifications: This includes:
  • Regular physical exercise to reduce stress
  • Well-balanced diet to improve health
  • Managing stress effectively
  • Resting during flare-ups