Behcet Disease – Causes, Symptoms, and Treatment

Behcet’s disease or Behcet’s syndrome is a chronic remitting and relapsing inflammatory disorder characterised by recurrent oral ulcers, genital ulcerations and eye manifestations. It can often be associated with other systemic involvement.  

Causes of Behcet disease

The exact cause of Behcet’s disease is unknown. It is believed to be partly genetic. Environmental factors also play a role. It is most frequently associated with carriers of HLA-B51, who are at high risk for developing Behcet disease.

Age group and geographical pattern

Behcet disease usually onsets in the 30s and is rare before puberty or after 50 years.

The disease affects people of both sexes equally. The disease’s geographic pattern points to distribution along the historic “Silk Route,” with the middle and the far east having the highest incidence.

Symptoms of Behcet disease

Painful ulcerations in the mouth. They are called aphthous ulcers. The ulcers can be relapsing. Painful genital ulcerations can develop in the anus, vulva or scrotum.  

• Eyes – Eye involvement can be in the form of posterior uveitis, anterior uveitis or retina vasculitis. It can present as painful eyes, red eyes and decreased vision. Retinal vasculitis can cause painless loss of vision.  

• Gastrointestinal manifestations – Abdominal pain, abdominal bloating, nausea and diarrhoea with or without blood.

• Lungs – Chest pain, blood in sputum, cough or fever.  

• Others – Arthritis causing joint pain and swelling.

What is Neuro Behcet?

 Meningo encephalitis presents with fever, headache, altered sensorium, hemiparesis, hemi-sensory loss, seizures and cognitive changes.  

5% to 10% of patients with Behcet disease experience central nervous system involvement. 80% of these cases are caused by parenchymal involvement, most frequently of the brainstem. This results in cerebellar, pyramidal and sensory signs and symptoms.

How is Behcet disease diagnosed?

There is no single laboratory test that can diagnose Behçet’s disease. There is an international study group criteria for the diagnosis of Behcet disease.

According to the criteria, the patient must have oral ulcers (that recur thrice over a period of 1 year) along with 2 of the following 4 criteria.

1. Eye inflammation  

2. Genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)

3. Pathergy reaction – minor trauma to the skin leads to the development of skin lesions. The pathergy test has an accuracy of 95 per cent to 100 per cent, but the results are often negative in European and American patients.

4. Skin lesions  

How is Behcet disease treated?

No definitive treatments are available to cure the disease. There is treatment available to control symptoms and inflammation.

Corticosteroids suppress inflammation. Immunosuppressants such as methotrexate, azathioprine, cyclophosphamide and cyclosporine are used as steroid-sparing agents. Biologics available are used in more severe cases.   

Conclusion

Behcet’s syndrome is a rare disorder. The occurrence of the disease in India is very low. Combined treatment  methods are used if a case is diagnosed. Research holds the future definitive diagnostic and treatment options for Bechet’s disease and its variation.

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