In the medical literature, Behçet's disease is often called Touraine's aphthosis major or Adamantiad syndrome. This rare disease belongs to the category of systemic chronic pathologies with unknown causes. The peculiarity of this defect lies in the fact that several types of symptoms are simultaneously observed with it. Although, against the background of a pronounced clinical picture, other anomalies can be identified.
Most often, Behçet's disease occurs in Japan and Mediterranean countries, among which it is worth highlighting Turkey with a very unusual cuisine. Tourists from other areas do not always notice differences in food habits. In addition to belonging to the indigenous populations of the Mediterranean and Japan, people who consume too many walnuts should be considered at risk.
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General information
Initially, the description of Behcet's syndrome included the mandatory presence of several symptoms:
- iritis - inflammation of the iris;
- hypopyon - purulent contents in the lower part of the eye;
- ulcers on the mucous membranes of the genitals and oral cavity.
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The disease owes its name to the Turkish dermatologist Behçet, who actually described its features and proposed classifying it into a separate nosological group. A few years earlier, this pathology was studied by another Greek doctor, Adamantiad. That is why some sources mention the double name of the disease - Behçet-Adamantiad syndrome.
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True, in fact, the first information about such a rare vice is found in the works of Hippocrates from 377 BC. It was this scientist who described eye damage, causing blindness, accompanied by ulcerative injury to the skin and mucous membranes, mainly in residents of Asia Minor.
According to the ICD, Behçet's disease is coded M35.2.
Symptoms
Behcet's syndrome manifests itself differently in each individual patient. In some patients, symptoms are mild, while in others they can be much more severe. Severe symptoms usually occur months to years after the disease first appears. Typically, symptoms come and go in alternating periods. The four most common symptoms are: ulceration in the mouth and genitals, inflammation of various parts of the eye, and arthritis.
Oral ulcers are usually the first symptom observed in patients with Behçet's syndrome; they usually occur in all patients and may precede the onset of other symptoms. The sores usually have a reddened edge and appear several at a time. They are usually painful and interfere with eating. Their sizes vary from a few mm to 20 mm in diameter; most often they are located on the gums, tongue and oral mucosa. The ulcers usually heal on their own within 10-14 days, but they soon reappear. Small ulcers heal without scarring, while larger ones usually leave them behind.
Genital ulcers occur in more than half of patients with Behçet syndrome; most ulcers are observed on the scrotum in men and the vulva in women; they look like ulcers located in the mouth and can be painful. Ulcers usually cause scarring.
Inflammation of the middle layer of eye tissue is called uveitis and occurs in more than half of patients suffering from Behçet's syndrome. This symptom is more common in male patients and usually appears during the first two years of the disease. Inflammation of the deepest layer of the eye (the retina) is called retinitis and is less common. Inflammation of the eyes can cause blurred vision and, less commonly, pain and redness. A common inflammation of the eye tissues can cause partial or complete loss of vision, which requires the intervention of an eye doctor to prevent.
Joint inflammation occurs in more than half of patients. Arthritis causes pain, swelling and stiffness in the affected joints, most commonly the knees, ankles, wrists and elbows. It usually lasts for several weeks and does not cause permanent damage to the joints.
In addition to the most common symptoms, Behçet's syndrome can cause skin lesions, intravascular coagulation and inflammation of the veins, inflammation of the central nervous system and digestive organs.
Skin lesions appear as red, slightly painful, vertical nodules (erythema nodosum) that appear spontaneously on the front of the thighs or upper torso.
Vascular symptoms include acute inflammation of the wall of venous blood vessels, which develops in approximately 10% of patients. This usually causes the formation of intravascular blood clots (thrombophlebitis), most often in the veins of the lower extremities. Thrombophlebitis causes pain, tension and often swelling of the affected limb.
The rupture of an intravascular thrombus and its release into the bloodstream can lead to extremely serious and fatal complications such as pulmonary thromboembolism. A small proportion of patients may experience arterial damage.
CNS damage occurs in approximately 10% of patients with Behçet's syndrome. Most often, damage manifests itself in the form of meningoencephalitis - inflammation of the brain and the thin membranes that cover and protect it. Meningoencephalitis causes fever, severe headache, nausea, vomiting, stiff neck, irritation from noise and light, difficulty coordinating movements, weakness of certain muscle groups, and less commonly, confusion or coma. This condition is very serious and without medical intervention can lead to permanent damage to the central nervous system, as in a stroke. Typically, CNS involvement occurs late in the course of the disease, most often years after diagnosis.
Damage to the gastrointestinal tract is manifested by the development of inflammation and the formation of ulcers in the digestive tract (most often in the small and large intestines), causing abdominal pain, diarrhea or constipation, vomiting or bleeding.
At-risk groups
Taking into account the fact that the pathology occurs most often in the territory between the Mediterranean and Asia, it is also often called the Silk Road defect. In other areas, cases of Behcet's disease are recorded extremely rarely.
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The disease is most common in Turkey, followed by Japan, Saudi Arabia, Iran and Korea. In other countries, the incidence is only one case per 100 thousand people.
Behçet's disease is diagnosed much more often in men than in women. The only exceptions are Japan and Korea - here the pathology mainly affects the female population. The first signs usually appear in patients over 40 years of age.
Causes
The cause of this condition is currently being studied, with evidence suggesting a significant association of the disease with the HLA-B51 gene, which is observed in some ethnic groups.
Autoimmune causes that have been previously proposed cannot be confirmed due to lack of evidence of autoimmune bodies during illness.
People between 20 and 40 years of age are most often affected. It is detected more among people with severe forms. This condition is rare in the United States and occurs in a less severe form among women.
Etiology of the disease
The specific causes of Behcet's disease have not yet been identified. Today there are two theories of the appearance of pathology: genetic and infectious. Taking into account the fact that the defect is much more common in older people, it is likely that hereditary predisposition in combination with external harmful factors plays an important role.
Genetically, people who have HLA-B51 antigens and abnormal levels of cytokines IL-6, 17, 8, 21 are more susceptible to the disease.
As for the infectious theory of the development of the disease, the main place in it is given to streptococci, herpetic viruses and mycobacterium tuberculosis.
Among other things, the appearance of this defect can be influenced by prolonged abuse of alcohol, drugs, tobacco, and the presence of autoimmune pathologies, which are accompanied by damage to the mucous membranes.
Danger of the syndrome
What consequences can Behcet's disease have for the patient? The syndrome can lead to:
- generalized aphthous stomatitis;
- deep ulcers on the mucous membranes of the vagina, penis, mouth, scrotum;
- large scars instead of ulcers.
If a bacterial infection occurs, the resulting ulcers may fill with pus. Skin symptoms of the disease are characterized by the occurrence of papulovesicular rashes, vasculitis of varying severity, erythema nodosum, purulent pustules, and pyoderma. Many patients also experience hair loss in the form of stellar alopecia.
If joints are involved in the pathological process, symmetrical polyarthritis occurs, which is characterized by the absence of destructive changes.
The appearance of uveitis provokes blurred vision, photophobia, chronic lacrimation, fogginess and blurred images. The development of this condition can lead to complete blindness.
Diagnostics
Behçet's disease can be suspected based on at least three existing symptoms, the main one of which is aphthous stomatitis. Examination, questioning and ordering of necessary tests are within the competence of a rheumatologist. An important evaluation criterion is the pathergic test for reaction from injection.
All patients undergo a general and biochemical blood test, coagulogram and immune tests. The analysis results show:
- neutrophilic leukocytosis;
- moderate anemia;
- increase in ESR;
- increased fibrinogen, haptoglobin, alpha globulin, seromucoid;
- decrease in the level of T-lymphocytes;
- increased levels of circulating immune complexes (CIC), complement and class A immunoglobulins.
A coagulogram will show increased activity of blood clotting factors. If necessary, ultrasound of the joints, X-ray or arthroscopy, as well as diagnostic intra-articular puncture are prescribed.
Plasmapheresis is a modern method of blood purification, similar to bloodletting, which was used to treat people in past centuries. During the procedure, aggressive immune bodies are removed, which slows down the pathological process and relieves unpleasant symptoms
Possible consequences of the vice
Among other things, Behçet's disease may well be accompanied by damage to the digestive system, which makes differential diagnosis with Crohn's disease and nonspecific colitis more difficult.
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Some patients may experience:
- nonspecific arthritis;
- pleurisy;
- gangrene;
- thrombosis;
- amyloidosis;
- pulmonary embolism;
- myositis;
- renal failure;
- myopathies;
- schizoaffective mental disorders.
In women with Behçet's disease, vulvovaginitis regularly occurs, and in men - epididymitis. It is noteworthy that all these pathologies are recurrent in nature.
Causes and risk factors
In Behçet's disease, veins and arteries of medium and small size are mainly affected. An inflammatory process develops in the walls of blood vessels; they are infiltrated by lymphocytes, neutrophils (characteristic of intravascular conglomerates of neutrophils), monocytes, and plasma cells. As a result, the walls of the affected vessels thicken, causing them to narrow or completely obliterate, and blood circulation in them is disrupted. Violation of tissue trophism leads to various manifestations of the disease.
Prevention of Adamantiadis-Behçet disease has not been developed, since the causes and mechanism of its development are unknown.
The reasons that give rise to the pathological process are currently unknown. The influence of the following factors is expected:
- genetic mutations. The assumption is partly confirmed by the fact that the disease is most widespread among men in Asian and Middle Eastern regions;
- toxic factors and infections. They cause tissue damage to the walls of blood vessels, and then support the inflammatory process in them;
- autoimmune mechanisms. In the body of patients suffering from Behçet's disease, antibodies to the cells of the oral mucosa are detected, and the content of circulating immune complexes (CIC) is increased.
One of the possible causes of Behçet's disease is genetic mutations
Symptoms of Behçet's disease
Treatment of the syndrome and future prognosis largely depend on the initial manifestations of the clinical picture that worry the patient. After all, as already mentioned, pathology is often accompanied by many other diseases, which, in turn, give certain complications. That is why the patient needs high-quality diagnosis and comprehensive treatment not only of Behçet’s disease itself, but also of accompanying defects.
First of all, it is worth saying that ulcers that appear during illness in the mouth and on the skin are always recurrent. Soon the patient develops the same lesions, but on the mucous membranes of the genital organs. As the defect develops, the ulcers become more extensive; in addition, they can become infected, fill with pus and leave deep scars in their place.
In addition, patients complain of:
- severe visual impairment;
- pain, pain and burning in the eyes;
- the appearance of fog in vision;
- severe migraines.
Many patients also note a sign of morning stiffness in the joints and symmetrical swelling in them. The skin is likely to develop erythema nodosum, acne-like rashes, pustular neoplasms and pseudofolliculitis.
Treatment
There is currently no specific treatment for Behçet's syndrome. Therapy is aimed at relieving symptoms and discomfort, reducing their manifestations and preventing the development of severe complications such as disability due to joint damage or blindness. Treatment is individualized depending on the severity of the disease and the tissues and organs involved. It is carried out using various drugs and their combinations.
Painful ulcers in the mouth and genitals are treated with topical creams and pastes containing corticosteroids (cortisone and its derivatives). In case of mild to moderate eye damage, treatment is carried out by administering anti-inflammatory drugs in the form of drops or intraocular injections.
Nonsteroidal anti-inflammatory drugs reduce the symptoms of inflammation without affecting the mechanisms of autoimmune inflammation; they are used to treat mild to moderate arthritis, often in combination with other anti-inflammatory and immunosuppressive medications.
Corticosteroids are powerful anti-inflammatory drugs that relieve swelling, inflammation and pain throughout the body from severe joint damage, skin ulcers, eye inflammation and central nervous system inflammation. Corticosteroids are taken in the lowest possible dosage and for the shortest possible time in specific regimens due to the risk of serious side effects. Treatment with corticosteroids should be discontinued gradually due to the risk of exacerbation of disease symptoms.
Drugs that suppress the immune system relieve autoimmune inflammation throughout the body and reduce symptoms of the disease. This class of medications is used for serious vision and nervous system problems and can cause serious side effects. Their long-term use requires monitoring of blood parameters indicating the occurrence of side effects. Some of the most commonly used immunosuppressants are azathioprine (its side effects include bone marrow suppression), chlorambucil (can cause infertility), cyclosporine (can cause kidney and liver damage), cyclophosphamide, and methotrexate. Colchicine is a medicine used to treat gout and, in Behçet's syndrome, to treat inflammation of the skin and eyes.
Other signs
A common symptom of Behçet's disease is undulating fever and weight loss even with adequate nutrition. In the case of ulcerative formations on the mucous membrane of the digestive tract, bloody diarrhea is observed.
Behcet's disease in children, adult women and men proceeds almost identically. The only difference lies in the fact that representatives of the stronger sex in adulthood develop damage to the visual apparatus much faster and thrombophlebitis occurs more often.
Although the full range of signs of pathology is observed, as a rule, after 40 years, already in childhood patients are faced with regular aphthous stomatitis, systematic visual disorders, convulsive attacks, migraines, bowel disorders, and sometimes blood clots after bowel movements.
Men, among other things, may also complain of chronic swelling of the scrotum.
Diagnosis of Behçet's disease
Mandatory and one of the most important criteria for diagnosis is the presence in the patient of recurrent ulcerative lesions of the oral mucosa.
In addition, important symptoms for diagnosing this pathology also include:
- the presence of chronic ulcerations of the mucous membrane of the genital organs;
- complications in the activity of the visual system - keratitis, uveitis, iridocyclitis, iritis, hypopyon, retinal hemorrhages;
- dermatological defects - erythema nodosum, papulopustules, acne-like rashes and pseudofolliculitis.
As for less significant signs, it is worth noting the symptoms of injury to internal systems:
- ulceration of the mucous membrane of the stomach and intestines;
- nonspecific arteritis, pericarditis, myocarditis;
- symptoms of damage to the central nervous system - seizures and aseptic meningitis;
- symmetrical arthritis;
- kidney destruction;
- epididymitis in men.
It is noteworthy that in order to be diagnosed with Behçet's disease, the patient must exhibit all the important symptoms of the disease. If half of these signs are present, it can be considered probable.
Features of detection
A positive pathergy test is considered a highly specific symptom of the disease. It is positive if, 1-2 days after the subcutaneous injection, the patient develops a pustular rash in the injection area.
In addition, when pathology is detected, differential diagnosis with similar ailments is mandatory:
- Crohn's disease, malignant intestinal tumors, ulcerative colitis;
- herpetic stomatitis, genital herpes;
- reactive arthritis and ankylosing spondylitis;
- multiple sclerosis.
When performing diagnostics, the following results are taken into account:
- ultrasound examination of the abdominal organs;
- tests for tumor markers;
- fundus examinations;
- biochemical blood testing;
- immunological blood test;
- irrigoscopy;
- OAM;
- UAC;
- electrocardiograms;
- colonoscopy;
- radiography and ultrasound of joints;
- fibrogastroduodenoscopy.
Histological examination of biopsy specimens of ulcers formed in the patient is mandatory.
Among other things, the victim may be recommended to consult other specialists with a narrow focus: ophthalmologist, cardiologist, neurologist, gastroenterologist, nephrologist and others.
Therapy
It is impossible to completely get rid of this pathology. Behcet's disease is characterized by a chronic course, but complex therapy makes it possible to transfer the defect into remission and constantly monitor the occurrence of exacerbations. In this way, the patient's quality of life can be significantly improved.
Treatment of Behçet's disease involves the use of immunosuppressive and cytostatic medications. These drugs can be combined with glucocorticosteroids and Infliximab.
If severe arthritis is detected, it is advisable to use non-steroidal anti-inflammatory drugs, methotrexate and colchicine.
To eliminate severe damage to the digestive system, glucocorticosteroids, azathioprine, sulfasalazine, and TNF-alpha inhibitors are prescribed.
If there are appropriate indications, the patient may be additionally prescribed antibiotics and antiviral drugs.
