Behcet ‘s disease is a chronic disease of unknown cause, which is characterized by recurrent oral aphthae, genital ulcers and eye lesions (uveitis) for the first time by Turkish scientist Hulusi Behçet. Inflammation of the vein (vasculitis) lies at the root of the disease.
It is relatively more common in countries located on the old Silk Road line such as Turkey, Israel, Iran, North China and Japan around the world.
It mostly occurs in men aged 30-40 years.
The disease presents with periods of exacerbation and asymptomatic periods.
Depending on the involvement in Behçet’s disease, two forms can be mentioned:
- Vasculobehçet: Involves all veins and arteries in the body. Venous involvement progresses with deep vein thrombosis (vein occlusions), and arterial involvement with vasodilation, occlusion and inflammation.
- Neurobehçet: It progresses with increased intracranial pressure, stroke and other nerve involvement symptoms due to the involvement of brain vessels.
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Causes of Behçet’s disease:
It can be mentioned genetically. More than half of the patients are HLA B-51 carriers. There are studies stating that the symptoms are milder in the presence of HLA B-27. It focuses on the triggering of environmental factors along with genetic predisposition and autoimmune features. It is also claimed that microorganisms such as HSV-type1 streptococcus sanguis act as environmental triggers.
The autoimmune feature of the disease is emphasized. The immune system has waged war against normal body cells, especially vascular cells. This condition, the cause of which cannot be understood, leads to vascular inflammation (vasculitis) and organ involvement.
Symptoms of Behçet’s disease:
Mucosal and skin lesions are the most common findings at the onset of the disease. Initially, eye and vascular involvement is less common.
If there is organ involvement in a young patient, life-threatening is higher.
The main symptoms of Behçet’s disease are:
- Mouth ulcers: This is the first and most common finding. Wounds occur in the mouth, on the inside of the lips, on the cheek, gums, tongue, cheeks. The middle part is white, the edges are red, painful sores. It heals in a few weeks but recurs.
- Genital ulcer: A similar mouth ulcer is seen in the scrotum in men, and in the vulva and vagina in women. It is usually painful and heals with scarring. It is wider than mouth ulcers but recurs less frequently.
- Other skin findings: Erythema nodosum, acne-like nodules, painful and swollen fluid-filled blisters, necrotic and pus-filled lesions may be seen.
- Ocular findings: More than half of the patients have eye involvement and permanent vision loss occurs in a quarter of them. Chronic, recurrent uveitis is mostly seen in eye involvement. Vision loss develops due to optic nerve involvement. In addition, intraocular hemorrhages, glaucoma, cataracts can be seen.
- Joint involvement: It is seen in half of the patients. It manifests itself with joint pain, especially in the feet and knees.
- Vascular involvement: Although it affects almost all vessels, more vein involvement is seen. It starts with fever, weakness, and an increase in CRP values. It then progresses with vessel narrowing, enlargement, necrosis, and inflammation. It can involve the lungs, liver and all organs. Most of the deaths are due to lung involvement. In lung involvement, bleeding from the mouth and bloody sputum are seen.
- Nervous system involvement: It is seen in one tenth of the patients. The brain stem is involved. Loss of sensation in half of the body, confusion, sphincter dysfunction occurs.
- Gastrointestinal system involvement: Due to the inflammation of intestinal vessels, abdominal pain, nausea, vomiting, diarrhea and weight loss can be seen after meals. Gastritis, hepatitis, pancreatitis can be seen.
Diagnosis of Behçet’s disease:
There is no definitive diagnosis of Behçet’s disease. Diagnosis begins with a good history and physical examination. Oral and genital lesions, skin and eye findings are investigated. In the examination, signs and symptoms of the disease and organ involvement are investigated. An eye examination is done.
- Blood test: CRP, sedimentation, HLA typing are evaluated.
- Pathergy test: It is the most used diagnostic test. Sterile saline is injected under the skin, and within 1-2 days, redness or acne-like lesions are seen on the skin. The test does not provide information about the severity of the disease. It is not sufficient for diagnosis alone. It is evaluated together with other findings.
- Urine tests
- Imaging tests (direct X-ray, computed tomography, magnetic resonance imaging),
- Skin biopsy
Diagnostic Criteria:
In addition to mouth ulcers that develop at least 3 times within 12 months, at least two of the following signs and symptoms are required for diagnosis:
- Genital ulcers
- Skin lesions
- Eye findings
- Pathergy test positivity.
Treatment of Behçet’s disease:
There is no definitive treatment for Behçet’s disease. Early diagnosis is an important step in the treatment of the disease. The aim of treatment is to prevent inflammation in different organs and tissues.
The disease requires the coordinated work of different specialties (rheumatology, ophthalmology, neurology, dermatologist, dentist, gynecologist, surgeon, etc.).
Treatment options in the treatment of the disease:
- Drug therapy: A drug treatment plan is applied, usually in the form of a combination of different drugs. Drug treatment can be continued outside the exacerbation periods of the disease. Drugs used as drug therapy:
- Steroids: These are drugs with high anti-inflammatory properties. Topical or systemic corticosteroid therapy can be used in Behçet’s disease. Although steroid therapy is sometimes miraculously effective, side effects may occur, especially due to long-term systemic (oral, intravenous) drug use. These may be in the form of weight gain, high blood pressure, glaucoma, cataracts, weakening of the bones, and increase in blood glucose level.
- Immune system suppressants: The basis of Behçet’s disease is the attack of the immune system on body cells. Therefore, by suppressing the immune system, it is tried to prevent it from attacking its own cells. In this way, inflammation is prevented. Drugs that can be used for this purpose are:
- Colchicine (mostly first choice),
- Cyclosporine,
- Methotrexate,
- Azathioprine,
Some side effects of drugs that suppress the immune system can be seen. These are mostly as follows:
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- Increased risk of infection,
- Nausea, vomiting,
- Stomach ache,
- Impaired liver function,
- Disruptions in blood tests,
- Temporary hair loss,
- Congenital anomalies during pregnancy (should not be taken).
- Biological agents: Based on the auto-immunity feature of the disease, some drugs specific to immune system cells are also used in the treatment of the disease. For this purpose, drugs such as alpha interferon and infiliximab are preferred.
- Physical therapy: It may be necessary to reduce and prevent symptoms and signs, especially in patients with joint involvement.
- Psychological treatment: The disease is a long-term disease. The treatments given are not one hundred percent effective treatments. This situation complicates the life of the patient and disrupts his psychology. For this reason, it is necessary to support the patient psychologically and to have regular psychological check-ups.
- Surgical treatment: Surgical treatment options can be tried in large ulcers, skin lesions, some organ (intestinal, eye, etc.) involvements.
Prognosis of Behçet’s disease:
Behçet’s disease manifests itself as periods of exacerbation and silent periods.
The course of the disease varies depending on the location and severity of the disease. Ocular and neurological involvement is unfavorable in terms of prognosis.
The most common causes of death are large vessel inflammation and nerve involvement.
Early diagnosis and initiation of the treatment process are positive for the course of the disease.
Permanent damage due to joint involvement is not very common, but permanent damage can be seen due to eye and nerve involvement.