Behcet's Disease

Behcet's disease is a rare disorder with no underlying cause. It is usually considered an immune activity triggered by exposure to an agent. Most commonly occurs in a patient with HLAB51 genetic predisposition. It is a chronic disorder causing the occlusion of the arteries and veins of all sizes affecting the various organs of the body.

It is also known to be associated with some of the environmental factors which include, Herpes virus, hepatitis virus, bacteria, and pollutants. Most commonly occurs in the middle age group 30-40 years and is more often seen in males than females. Patients with Behcet’s disease show any particular antibodies as the other autoimmune diseases show

Behcet's disease was first described by a Turkish scientist Hulusi Behcet, hence the name. It classically presents as orogenital ulcers, eye inflammation, and mouth. The following image shows the classical lesions of Behcet’s disease.

Behcet's Disease: Causes

There is no known cause for the disease, but some studies have suggested the following causes −

• It may occur as an autoimmune reaction to the agent in which the body assumes some of the normal tissue of the body as a foreign body and initiates the reaction involving the blood vessels

• Increased prevalence among particular races and families indicates the genetic link more often associated with HLAB51.

• Exposure to infectious agents such as streptococcus, staphylococcus, and herpes simplex virus type 1, has also been studied as the cause of the Behcet's disease.

Behcet's Disease: Symptoms

The classical symptoms of Behcet’s disease include orogenital ulcers, inflammation of the uveal tissue of the eyes, and typical skin lesions.

• Aphthous Ulcers − More than 90% of the patients present with mouth ulcers which are usually painful, multiple, and recurrent. Ulcers can be present over the hard palate, soft palate, buccal mucosa, lips, and gums. Occurring more or equal to three times in a year gives the hint for diagnosing Behcet’s disease. Genital ulcers are also present in more than 80% of the patients. Usually seen on the area over the scrotum in males and over the area of the vulva in females. Almost all the ulcers heal without scarring.

• Skin Lesions − Behcet presents with erythema nodosum-like lesions which are pale in the center and surrounded by a reddish area around. More commonly seen in the lower parts of the body. Scratching over the skin with a mild sharp agent causes an erythematous reaction on the skin within 24 to 48 hrs this is called a pathergy test.

• Ocular Features − More than half of the patients show eye involvement present within inflammation of the uveal tissue of the eye (uveitis) which causes decreased vision, redness, and intolerance to light.

• Musculoskeletal − Pain in the joints especially in the knee joint is present, other joints like ankles, wrists, and elbows are also involved.

• Vascular − Inflammation of the arteries and the veins causes redness and swelling over the hands and legs when blood clotting is present, also can lead to narrowing and blockage of the vessels.

• Gastrointestinal − Patients may present with loose motions, pain in the abdomen, and bleeding.

• Central Nervous System − if inflammation involves the brain and the nervous system patient presents with headache, fever, disorientation, imbalance, or paralysis.

• Other organs like lungs, and kidneys can also be affected.

Behcet's Disease: Risk Factors

The probable risk factors of Behcet’s disease include −

• Age − More commonly seen in young adults between 20 to 40 yrs, can also occur in younger and old age also

• Race − seen more commonly in countries of the middle east and east Asia

• Sex − occurs in both men and women but is usually more severe in males

• Genetic − More common predisposition with HLAB51 and HLAB5

Behcet's Disease: Diagnosis

The diagnosis of Behcet’s disease is done clinically based on the presentation of the patient, there is a single laboratory test that confirms the diagnosis of Behcet’s disease.

Criteria for the diagnosis of Behcet’s disease include (at least two of the following should be present) −

• Orogenital ulcers − Recurring at least 3 or more times in a year

• Inflammation of the eyes(uveitis)

• Typical skin lesions

• A positive pathergy test: This indicates the autoimmune reaction, scratching over the skin with mild sharp instruments induces a typical erythematous reaction over the skin without 24 to 48 hrs

Behcet's Disease: Treatment

The mainstay of treating patients with Behcet’s disease include

• Corticosteroids, most commonly prednisolone is used. These suppress inflammation and the autoimmune activity

• Colchicine, helps in patients with joint pain and also patients with orogenital ulcers and be benefitted

• Apremilast has been approved for treating mouth ulcers in patients of Behcet’s disease

• Immunosuppressant Drugs such as methotrexate, azathioprine, and cyclophosphamide can also be given

• Biological Agents like infliximab, etanercept, and tocilizumab also play the role

Behcet's Disease: Prevention

There is no prevention for Behcet’s disease occurrence, but the aim should be to prevent the organ damage. Diagnosing the disease at the earliest and treating the disease early can prevent the involvement of organs and the complications.

Some of the studies have also shown that a healthy diet and having good immunity power can also reduce the severity of the presentation of the disease.

Conclusion

Behcet’s disease is a rare disorder involving the arteries and veins of unknown cause. It is known to be autoimmune-mediated. There is no confirmatory test for the disease but based on the presence of clinical features can be diagnosed early. Since the disease presents involving the various parts of the body it needs to be assessed by specialists such as ophthalmologists, rheumatologists, dermatologists, cardiologists, and neurologists

There is no known cure for the disease but can be prevented by getting serious by treating the disease at the earliest. The mainstay of the treatment includes corticosteroids, immunosuppressants, colchicine for joint pains, and oral apremilast approved for ulcers.