Behçet’s disease, also known as Behçet’s syndrome, is a rare, chronic, and complex inflammatory disorder that affects multiple parts of the body, including the eyes. Named after the Turkish dermatologist Hulusi Behçet who first described the condition in 1937, this disease can cause significant ocular complications, leading to vision impairment or loss if not properly managed. This article delves into the ocular manifestations of Behçet’s disease, including its symptoms, diagnostic methods, and treatment options.

Behçet’s disease is systemic vasculitis, which means it causes inflammation of the blood vessels throughout the body. The exact cause of Behçet’s disease is unknown, but it is believed to result from a combination of genetic and environmental factors. The disease is more prevalent in regions along the ancient Silk Road, including countries in the Middle East, Asia, and the Mediterranean.

When Behçet’s disease affects the eyes, it is referred to as ocular Behçet’s disease. The ocular manifestations are among the most serious complications of the disease, as they can lead to significant morbidity. The primary ocular symptoms include:

1. Uveitis: This is the most common ocular manifestation, occurring in about 70-90% of patients with Behçet’s disease. Uveitis is an inflammation of the uvea, the middle layer of the eye, which includes the iris, ciliary body, and choroid. It can be anterior (affecting the front of the eye), posterior (affecting the back of the eye), or pan-uveitis (affecting the entire uvea).
2. Retinal Vasculitis: This condition involves inflammation of the retinal blood vessels and can lead to retinal damage, vision loss, and even blindness if not treated promptly.
3. Hypopyon: This is a condition where pus accumulates in the anterior chamber of the eye, causing pain, redness, and blurred vision.
4. Conjunctivitis: Inflammation of the conjunctiva, the membrane covering the white part of the eye, can cause redness, irritation, and discharge.
5. Corneal Ulcers: Open sores on the cornea can develop, leading to pain, tearing, and sensitivity to light.

Diagnosing Behçet’s disease, particularly when it affects the eyes, can be challenging due to its rarity and the variability of symptoms. There is no specific test for Behçet’s disease; instead, diagnosis is based on clinical criteria and the exclusion of other conditions. Key diagnostic criteria for Behçet’s disease include:

1. Recurrent Oral Ulcers: Painful mouth sores that occur multiple times within a year are a major diagnostic criterion.
2. Genital Ulcers: Recurrent painful sores on the genitals.
3. Skin Lesions: Erythema nodosum (painful red nodules usually found on the legs) or acne-like lesions.
4. Pathergy Test: A hypersensitivity skin reaction to a minor injury or needle prick.
5. Ocular Involvement: Presence of uveitis or retinal vasculitis as described above.

In addition to these criteria, ophthalmic evaluation is crucial. An eye examination by an ophthalmologist can detect inflammation and other changes in the eye that are indicative of Behçet’s disease. Imaging studies such as fluorescein angiography and optical coherence tomography (OCT) can help assess the extent of retinal involvement.

The treatment of ocular Behçet’s disease aims to reduce inflammation, prevent relapses, and preserve vision. A multidisciplinary approach involving rheumatologists, ophthalmologists, and other specialists is often required. Treatment options include:

1. Corticosteroids: These anti-inflammatory medications can be administered as eye drops, oral tablets, or injections to reduce inflammation quickly. However, long-term use can have significant side effects.
2. Immunosuppressive Agents: Medications such as azathioprine, cyclosporine, and methotrexate are commonly used to control the immune response and reduce inflammation. These drugs help prevent relapses and maintain remission.
3. Biologic Agents: Newer treatments like tumor necrosis factor (TNF) inhibitors (e.g., infliximab, adalimumab) and interleukin inhibitors (e.g., tocilizumab) have shown promise in managing refractory cases of ocular Behçet’s disease. These agents target specific components of the immune system to reduce inflammation.
4. Surgery: In severe cases, surgical interventions such as vitrectomy (removal of the vitreous gel from the eye) may be necessary to treat complications like retinal detachment or persistent inflammation.
5. Supportive Therapies: Regular monitoring, protective eyewear, and lifestyle modifications (such as avoiding triggers that exacerbate symptoms) are also important in managing the disease.

Living with ocular Behçet’s disease requires ongoing medical care and lifestyle adjustments. Patients should adhere to their prescribed treatment plans, attend regular follow-up appointments, and promptly report any changes in their symptoms to their healthcare providers. Support groups and counseling can also provide valuable emotional support and coping strategies.

Behçet’s disease is a challenging condition that can significantly impact ocular health. Early diagnosis and comprehensive management are crucial to prevent serious complications and preserve vision. By understanding the symptoms, seeking timely medical care, and adhering to treatment plans, individuals with ocular Behçet’s disease can lead fulfilling lives while effectively managing their condition.

World Eye Care Foundation’s eyecare.live brings you the latest information from various industry sources and experts in eye health and vision care. Please consult with your eye care provider for more general information and specific eye conditions. We do not provide any medical advice, suggestions or recommendations in any health conditions.