Behcet’s disease

Summary

Behcet’s disease is a chronic, multisystem autoimmune disease involving inflammation of blood vessels, called vasculitis, throughout the body. It is a rare disease, most commonly found in the Eastern Mediterranean countries and in eastern Asia. It affects more young men than women in those countries, but in the US it affects more women, most often in their 20s and 30s. The central nervous system, heart, and intestinal tract may be involved. Because this disease is so rare and it’s symptoms overlap those of other diseases, it may be very difficult to diagnose. Spontaneous remission may occur, which can add to the difficulty in diagnosis…The disease can lead to numerous signs and symptoms that can seem unrelated at first. They can include mouth sores, eye inflammation, skin rashes and lesions, and genital sores.

(Autoimmune Association, 2023)

Symptoms

Behcet's disease symptoms vary from person to person, can come and go or become less severe over time. Signs and symptoms depend on which parts of your body are affected.

Areas commonly affected by Behcet's disease include:

• Mouth. Painful mouth sores that look similar to canker sores are the most common sign of Behcet's disease. They begin as raised, round lesions in the mouth that quickly turn into painful ulcers. The sores usually heal in one to three weeks, though they do recur.

• Skin. Some people develop acnelike sores on their bodies. Others develop red, raised and tender nodules on their skin, especially on the lower legs.

• Genitals. Red, open sores can occur on the scrotum or the vulva. The sores are usually painful and can leave scars.

• Eyes. Inflammation in the eye (uveitis) causes redness, pain and blurred vision, typically in both eyes. In people with Behcet's disease, the condition can come and go.

• Joints. Joint swelling and pain often affect the knees in people with Behcet's disease. The ankles, elbows or wrists also might be involved. Signs and symptoms can last one to three weeks and go away on their own.

• Blood vessels. Inflammation in veins and arteries can cause redness, pain, and swelling in the arms or legs when a blood clot results. Inflammation in the large arteries can lead to complications, such as aneurysms and narrowing or blockage of the vessel.

• Digestive system. A variety of signs and symptoms can affect the digestive system, including abdominal pain, diarrhea and bleeding.

• Brain. Inflammation in the brain and nervous system can cause headache, fever, disorientation, poor balance or stroke.

(Autoimmune Association, 2023)

Diagnostic Criteria

Under Investigation

Study Classification Criteria

Under Investigation

Diagnostic Tests

Under Investigation

Organized Autoimmunity

(Alternative Autoimmune Disease Classification: FIEM, MIEM or BIEM, or FEM, MEM or BEM)

1. sex predominance (is an autoimmune disease primarily found in genetic Females, Males, or equally in Both?)

   1. Under Investigation

2. Inherited and acquired gene variations that cause increased susceptibility

   1. Human Leukocyte Antigen (HLA) Associations

      1. Under Investigation

   2. Other Gene Variations (mutations)

      1. Under Investigation

3. Gene Triggering Environmental Exposures

   1. Infections

      1. COVID-19 (Chang et. al, 2023, found a significantly higher risk of Behcet’s disease following COVID-19 infection in a study of 3,814,479 participants)

   2. Toxins

      1. Under Investigation

   3. Stress

      1. Needs to be assessed for each patient

4. Multiple interactive and destructive immune system pathologies

   1. Under Investigation

Tissue-Type or Cell-Type Attacked

Under Investigation

Treatment(s)

Under Investigation

Managing Specialist(s)

Under Investigation

Research Authors

Under Investigation

Research Institutions

Under Investigation

Average Time from Symptom Onset to Diagnosis

Under Investigation

Last Updated

September 05, 2023

References

Behcet’s disease. Autoimmune Association. (2023, April 22). Retrieved September 05, 2023 from https://autoimmune.org/disease-information/behcets-disease/

Chang R, Yen-Ting Chen T, Wang SI, Hung YM, Chen HY, Wei CJ. Risk of autoimmune diseases in patients with COVID-19: A retrospective cohort study. EClinicalMedicine. 2023 Feb;56:101783. doi: 10.1016/j.eclinm.2022.101783. Epub 2023 Jan 10. PMID: 36643619; PMCID: PMC9830133.