Behcet's Disease

Treatment of Behcet's disease is a possible disease ...


What is it?

Behcet's syndrome or Behcet's disease (BSE), recurrent oral (mouth) and genitals (sex organs) with ulcers, eye, skin, joints, leading to the vascular and nervous system involvement, the cause is unknown vasculitis (blood vessel ltihab a). BSE, in 1937, a Turkish doctor, Prof. Dr. Was described by Hulusi Behçet.

How often?

BS is more common in some parts of the world. The geographical distribution of BS coincides with the historical Silk Road. Japan, Korea, China, Iran, Turkey, Tunisia and Morocco, such as the Far East, mostly seen in countries in the Middle East and the Mediterranean. The prevalence in the adult population in Japan 1 / 10,000 and Turkey 1-3 / 1000. In Northern Europe the number of 1 / 300,000 is around.

Few cases have been reported from the USA and Australia. BS in children is rare, even in high-risk populations. Diagnostic criteria, the BS is completed before the age of about 3% of patients 16. The average age of onset of the disease 20-35't. Is equally distributed amongst both sexes, but males have a more severe.

What are the causes of the disease?

The cause of the disease is unknown (idiopathic). Genetic predisposition may play a role in the development of BS. There is no known trigger. Research into the causes of the disease and treatment is carried out in several centers.

Is it inherited?

There is no definitive way of inheritance, but a genetic predisposition said. In particular, the Far East and Mediterranean origin in patients with a genetic predisposition (HLA-B5) is associated with. Some familial cases described in the literature available.

Why was my child is sick?Avoidable?

The cause is unknown, and the block is not possible.

Is it contagious?

It is not contagious.

What are the main symptoms?

1) Mouth sores (ulcers): These lesions are almost always present. Mouth ulcers are the initial findings in two thirds of patients. Most children, childhood wounds that are indistinguishable from recurring mouth sores develop numerous small. Large ulcers are more rare, but the treatment is very difficult.

2) Genital ulcers: In boys the ulcers are mainly on the scrotum and, less frequently, is located in the penis. These almost always leave a scar in adult male patients. Girl children are influenced mainly external genitalia. These ulcers resemble the oral ulcers. Pre-pubertal children are seen less genital ulcers. In boys recurrent orchitis (inflammation of the ovaries) can be seen.

3) Skin involvement: There are different skin lesions. Acne (pimples) like lesions are present only after puberty. Erythema nodosum, usually located at the bottom of the legs, red, painful, raised sharply from the skin lesions. These lesions are more frequent among prepubescent children. "Pathergy reaction", a BS patients

response of the skin to the tip of the needle is dipped. This can be used as a diagnostic test in BS. After a sterile needle dipped into the skin of the forearm, a papule or pustule within 24-48 hours.

4) Eye involvement: This is one of the most serious manifestations of the disease. The prevalence in the community, although it is 50%, up to 70% interest in boys. Girls are affected less. The disease is bilateral in the majority of patients. Generally, the onset of eye syndrome is kept within 3 years. Eye disease is chronic, with occasional flares. Eye kept both front and rear cabins (anterior and posterior uveitis) After each flare, some structural damage occurs to cause gradual vision loss.

5) Joint involvement: about 30-50% of the children with BS joint involvement occurs. Generally, ankle, knee, wrist and elbow joints are affected. Monoarticular (single joint involvement) or oligo (4 or less joint involvement) is observed. This inflammation usually last a few weeks and without symptoms. Arthritis of BS to cause joint damage is very rare.

6) Neurological involvement: with BS seen Neurological involvement is rare in children. Seizures, increased intracranial pressure and brain associated with headache symptoms are characteristic. The most severe forms are seen in males. Some patients may develop psychiatric problems.

7) Childhood BS in vascular involvement is seen in 12-20%. May be an indication of deterioration. Usually the large blood vessels in the body. The most commonly affected are the veins of the calf. The calf is swollen and painful.

8) Gastrointestinal involvement: This is especially common in patients from the Far East. Ulcers in the intestine examination (sores) can be seen.

The disease the same in every child?

Each child is not the same. Some may have mild disease with oral ulcers, skin lesions may be some; in others eye or nervous system involvement. Also, there are differences between boys and girls. Often in men, with more eye and vascular involvement and more severe disease course is observed.

Disease in children than in adults, is it any different?

BS is rare in children compared to adults. Some differences are seen with the transition to adolescence. Disease in post-pubescent children is more like the adult disease. Common in children than in adults are more familial cases. Despite some exceptions, in general, similar to BSE adult disease in children.

How is it diagnosed?

The diagnosis is mainly clinical. To fill the international criteria described for BS patients can take 1-5 years. Diagnosis is usually delayed an average of 3 years. BS is no specific laboratory findings. Approximately half of the children carry HLA-B5. This is associated with more severe disease. Defined earlier pathergy skin test is positive in about 60-70% of patients. Or to appear as the vessel is required to identify the specific brain vascular or nervous system involvement. BS is a multi-systemic disease of the eye specialist, dermatologist, neurologist should be involved in the treatment of patients.

What is the importance of tests?

1. Pathergy skin test is important for diagnosis. The International Study Group classification criteria for Behçet's Disease is also included. The inner surface of the forearm with a sterile needle dipped 3-5 times. It hurts very little. The reaction is assessed 24-48 hours later. This extreme sensitivity of blood is drawn, or after surgery can also be seen in patients, therefore, patients with BS should not undergo unnecessary interventions.

2. Some blood tests are done for differential diagnosis, but there is no specific laboratory examination for BSE. Tests that show inflammation in general, are mildly elevated. In this test an increase in moderate anemia and white blood cells can be detected. If the patient is not being monitored in terms of disease or drug side effects, there is no need to repeat these tests.

3. Vascular or a number of imaging techniques applied to children with neurological involvement.

How long disease?

Usually the course of the disease includes periods of remissions and exacerbations. Disease activity decreases over time.

Long-term consequences of the disease (prognosis) What? Childhood is not enough data on long-term follow-up of BS patients. Based on the data we have do not require treatment can say that many patients with BS. However, eyes, nervous system and children with vascular involvement require special treatment and follow-up. Young men, in a way they had more severe disease than girls. The eyes are kept in the first few years of the disease. In particular, some breeds (eg. Japanese), as shown, vascular involvement (rupture of pulmonary arterial or other aneurysms), severe central nervous system disease, BS can be fatal in rare cases with intestinal ulcers and perforations. Long-term problems (morbidity) is a major cause of eye disease can be very severe. The child's growth may be retarded, mainly secondary to steroid therapy.

Full recovery possible?

Some mild cases may recover, but is often seen in most long-term recovery period.

How the disease can affect the daily lives of children and families? As with any chronic disease, BS does affects the child and family's daily life. If the disease is mild, without eye and other major organ involvements, usually the family may lead a normal life.

In this group, the most common problem with recurrent oral ulcers, so that might be a problem in most children. These lesions may prevent eating and drinking painful. Eye involvement may also be a serious problem for the family.

You can go to school?

The continuing education of children with a chronic disease is required. In BS if there is eye or other major organ involvement, the children can go to school regularly. Decrease in vision may require special education programs.

What about sports?

If only the skin and mucosal involvement may participate in sports if the child in question. During attacks of joint inflammation, sports should be avoided. Arthritis in BS is short-lived and resolves completely. Once the inflammation is the patient can resume sports. However, children with eye and vascular problems have to limit their activities. Prolonged in patients with vascular problems in the lower extremities is not recommended for survival.

Nutrition should be?

There are no restrictions with regard to food intake.

Climate can affect the course of the disease?

No, there is no significant effect on the course of the climate BS.

Children can be vaccinated?

Physician should decide about which vaccines the child can receive. If the patient is being treated with immunosuppressive drugs (corticosteroids, azathioprine, cyclosporine A, cyclophosphamide, anti-TNF) with live attenuated virus vaccines (measles, measles, mumps, oralpolio) vaccination should be postponed. Not contain living viruses, but only infectious proteins (tetanus, diphtheria, polio Salk, hepatitis B, pertussis, pneumococcal, haemophilus, meningococcus) can be applied.

Sexual life, pregnancy and birth control how it should be?

One of the major problems with sexual life is the development of genital ulcers. They can be recurrent and painful and interfere with sexual intercourse. Females with BS disease can lead a normal pregnancy have mild. If birth control applications they use immunosuppressive drugs are required. Therefore, to consult their physician about birth control and pregnancy.

How is eye involvement in Behçet's disease, what are the symptoms?

Ocular involvement anterior uveitis of Behcet's disease, posterior uveitis or anterior and posterior uveitis seen one "panuveitis" shape. Eye involvement occurs in patients with uveitis symptoms described above. These symptoms "hypopyon" gets a glimpse of where we are more common in the patients with Behçet's disease and indicates the possibility of severe damage to leave in style watch

What is the frequency of ocular involvement in Behçet's disease, and when does it occur?

Eye involvement in Behçet's disease occurs in approximately 45-55% of patients. This is because it is compared with the data obtained from patients admitted to the hospital does not reflect the true rate in the society. Behçet disease sometimes be accompanied by the onset of eye diseases. The reason for patients to other systems is the lack of evidence, forcing to go to the doctor. After a period in patients diagnosed with symptoms caused by other system involvement can occur in the eye, this ratio is quite low. Eye involvement may continue reviewing the same starting one eye. However, the disease is often both eyes.

Behçet's disease who are at high risk of ocular involvement and violence?

Undergo severe uveitis with the appearance of the eye's ocular involvement in Behçet's disease and illness and in this context is associated with severe vision loss lead to the onset of the disease at an early age and male gender. Eye involvement is more common in men than in women. Have eye involvement is also a sign of serious illness will watch. Eye disease is not the most important sign of severe exacerbations and de-activation of the findings we will watch often and heavily damaged leave in style in style watch.

How to watch ocular involvement in Behçet's disease and how factors such as uveitis alerts?

The frequency and severity of attacks, which is a feature of the clinical appearance of eye involvement shows differences from patient to patient. Lost come out of these attacks normal tissues and structures of the eye can cause permanent damage to development; which lead to permanent vision loss, the most important prognostic evaluation of uveitis. There is no sign of the emergence of a pre-uveitis. The disease is very well followed in arranging treatment according to clinical changes observed during the follow-up, making the protocol of treatment and transition to dual therapy fails to provide the parameters of protocols is important. Stress, fatigue, irregular use of drugs can induce uveitis and inflammatory diseases. In particular way, and suggested that the use of drugs on a regular basis can lead to exacerbation of the disease. Considering the size of the treatment should be continued to warn of the event done correctly.

Must treat all patients with ocular involvement in Behçet's disease?

Eyepiece "lens" behind you vitreous that fills the vitreous cavity inflammation "vitritis" is known as. Eye can only be included as a treatment for patients with symptoms vitritis. Topical eye drops and ointments containing corticosteroids are given for the suppression of inflammation in patients with anterior uveitis. Separation of adhesions that may occur between the lens and the iris and pupil of the objective (the pupil) expander drops are given at specified frequency. Adhesions in patients with high risk of developing the pupil expands as strong drugs is given by more frequent intervals. In the patients with posterior or panuveitis certain doses of systemic corticosteroids and immune systems suppressed by drugs administered alone or in combination, and severity of the disease. Such as systemic corticosteroids and immune system suppressants large vessel involvement outside of the eye disease and is also used in severe forms of neurological involvement.

What is the risk of vision loss in Behçet's disease?

During episodes of decreased vision in the eye tissue damage occurring as a result of temporary occurs. Recurrent and severe attacks after the cause of the damage caused, especially in the eyes of the backing layer is composed of permanent vision loss. Eye involvement is not seen as the center of the devastating and / or long-term vision can be maintained at a good level in female patients. More than made up for the risk of vision loss in countries such as Turkey and Japan due to the higher number of Behçet's disease. The disease is less common than in countries such as North America.

How to follow up patients with ocular involvement in Behçet's disease?

Eye involvement of patients with eye disease clinical departments of the uvea is periodically monitored regularly. Patients are asked to be aware of the emergence of new symptoms and control exacerbations except in case of doubt the development of the clinical eye examination is recommended to apply even if they are followed in an emergency. Follow the course of the disease to come regularly to control examination of the patients, the shape of the medication, to determine the dose and side effects, and is important for the determination of the complications of the disease.