Behcet's disease manifests itself in different clinical symptoms: symptoms of aphthous stomatitis, erythema nodosum, uveitis, arthritis, pulmonary, renal, gastric dysfunction, thrombosis, nervous system diseases, lesions bowel. for chronic pathologies, with periodic change of exacerbation and remission. May develop complications in the affected organs.
What it is?
Behcet's disease - systemic vasculitis of unknown etiology, characterized by a primary lesion of the mucous membranes of the eyes, mouth, skin and genitals
Statistics
Behcet's disease most commonly occurs in the territory of the Silk Road from Central Asia to China. In Turkey, the highest incidence - 420 cases per 100 000 population. In other countries - 1 in 15 000-500 000 people. men are affected more often than women (5: 1) in the Middle East, in Europe, on the contrary. The average age of disease manifestation - 25-30 years. In an earlier age in the development of Behçet's disease most commonly affects the eyes.
Men more likely to develop serious complications artery rupture, thrombosis, CNS, and women more likely to develop skin lesions.
Causes of
The exact etiology of the disease is not yet clear, but still he spoke of his Behçet viral origin. Subsequently, a specific virus was isolated from the blood of patients, but direct evidence that he is the cause of the syndrome, yet. There are theories that the causative agents of herpes and tuberculosis may precede aggravating circumstances and course of the disease.
In the last decade, discussed autoimmune mechanism of its development: patients often exhibit antigens HLADRW52i B51, which is especially great risk of Behcet's syndrome in the presence of these two antigens at the same time. In addition, reported cases of family illness and regional nature of talk about the role of genetic factors in the development of the disease.
The prevalence of Behcet's disease in men and women is not uniform: in the Middle East, it affects mainly men, but in Japan and Korea among patients women prevail. The age of most patients does not exceed 30-40 years.
The symptoms of Behcet's disease
Behcet's disease clinical picture is polymorphic symptoms as pathological changes occur throughout the body (see. Photo).
With the defeat of the eye occur:
- Uveitis. Inflammation of the choroid can be anterior (iritis and iridocyclitis) and rear when in the process involves the retina.
- Conjunctivitis - inflammation of the conjunctiva.
- Keratitis - inflammation of the cornea.
- Atrophy of the optic nerve and blindness - in severe cases.
skin and mucous defeat manifested by the following symptoms:
- Aphthae (small ulcers) in the mouth, which may coalesce to form large wound surfaces on the mucosa. The course of this protracted stomatitis, relapses 3-4 times a year.
- Sores on the penis, on the scrotum, genital lips.
- Nodular erythema - painful red knots in the legs and arms.
- Acne-like rash on the trunk and limbs skin.
The defeat of the nervous system with Behcet's disease always has severe manifestations:
- Inflamed meninges, or brain tissue (these diseases are called, respectively, meningitis and encephalitis).
- Increased intracranial pressure.
- There are disorders of the cranial nerves.
- Develop paresis.
- Deteriorating memory, dementia progresses.
On the part of the vascular bed following changes occur in Behçet's disease:
- There are signs of cutaneous vasculitis - hemorrhagic rash.
- There is thrombosis of large and small vessels.
- Formed arterial aneurysms.
It is because of vascular problems (such as thrombosis or aneurysm) develop deaths with Behcet's disease. The most dangerous are thrombosis and pulmonary artery rupture of an aortic aneurysm. When clogged leg arteries thrombi may develop gangrene.
In the pathological process may involve the large joints of the limbs and spine, while the patients complain of joint pain, impaired mobility. If autoimmune inflammation develops in the blood vessels of the lungs, the patient has hemoptysis, shortness of breath, pulmonary edema possible.
Kidney, gastrointestinal tract and heart with Behçet's disease affects less than already mentioned organs and systems, but the appearance of pathological symptoms from their side as possible. Patients may develop a peptic ulcer of the stomach and bowel disease, glomerulonephritis, vasculitis of the coronary vessels, myocarditis, pericarditis, etc.
complications
Complications of Behcet's disease appear when defeat comes to the central nervous system, internal organs and eyes. In this case, you may receive:
- glaucoma;
- atrophy of the optic nerve;
- loss of vision;
- paralysis;
- hearing loss;
- mental retardation;
If the disease is complicated by thrombosis, the condition can lead to gangrene of the extremities.
How to treat Behcet's disease?
When diagnosed with Behcet's disease carried out a comprehensive, long-term and systemic treatment. It is prescribed based on location of the lesion and the individual characteristics of the organism is strictly in the hospital. Since the etiology of the disease has not been established, only symptomatic treatment is carried out aimed at arresting the clinical symptoms and the prevention of complications.
Drug therapy comprises administering to patients medicaments to various pharmacological groups:
- Anti - "Ibuprofen", "Diclofenac" "Indomethacin"
- Antihistamines - "Suprastin", "Tavegil", "Tsetrin"
- Antiviral - "Acyclovir", "Isoprinosine"
- Antibacterial - "Azithromycin", "Cefazolin", "Tsoprofloksatsin"
- Vasodilators - "Pentoxifylline," "Niacin",
- Vitamins - "Aevit", "Oligovit"
- Vntikoagulyantov - "Aspirin", "Trental", "Kurantil"
- Arthrifuge - "Colchicine" "Allopurinol"
- Hormonal - "prednisolone", "Dexamethasone"
- Cytostatics - "Cyclophosphamide", "Methotrexate".
Topical treatments include: mouthwash and antiseptics treating ulcers, application of ointments with Gepatrombin, indomethacin, glucocorticoids, subconjunctival administration "Dexamethasone".
In severe cases, resorted to extracorporeal therapy: plasmapheresis, gemosobrtsii, ultraviolet irradiation, laser therapy, ultrasound, inductothermy. The use of surgical methods of treatment will help to eliminate the negative effects of the pathology.
prevention
Specific prevention against Behcet's disease does not exist, because it is not clarified its reasons. We can only highlight the main points that will help to support the immune system in the normal state:
- time to treat infectious diseases;
- arrest chronic infectious disease;
- follow the daily routine;
- regulate physical activity;
- abandon uncontrolled admission of drugs;
- strictly follow the doctor's prescription;
- to take measures to raise the immunity;
It is recommended to observe the hypoallergenic diet with high fiber content.
Forecast for life
Forecasts of Behcet's disease depend on the organs and systems that are involved in the pathological process. In the isolated form of the disease when struck by only the skin and mucous membranes, the prognosis is usually favorable.
In the case of uveitis, the patient's quality of life may be degraded partial or complete blindness, and most hazardous situations are CNS and blood vessels - in these cases, approximately 5% of patients die from the evolved complications.
