Behcet – s disease causes symptoms diagnosis how to treat

ARR – Association of Rheumatologists of Russia

BB – Behcet’s Disease

BPVP – basic anti-inflammatory drugs

GIBP – genetic engineering biological preparations

DNA – deoxyribonucleic acid

and TNF-? – tumor necrosis factor-alpha inhibitors

CT – computed tomography

MRI – magnetic resonance imaging

MRI – magnetic resonance venography

ICD 10 – International Classification of Diseases 10 Revision

ESR – erythrocyte sedimentation rate

CSF – cerebrospinal fluid

CRP – C-reactive protein

BDCAF (Beh? Et’s Disease Current Activity Form) – BB activity index

EULAR (European League Against Rheumatism) – European Anti-Rheumatic League

ISBD (International Society for Beh? Et’s Disease) – International Community for BB

ISGBD (International Study Group for Beh? Et’s Disease) – International BB Study Group

HLA – Human Leukocyte Antigens

Nasonov EL, editor. Rheumatology: Clinical recommendations. 2nd ed., Rev. and add. Moscow: GEOTAR-Media; 2010 s. [Nasonov EL, editor. Revmatologiya: Klinicheskie rekomendatsii. 752nd ed., Ispr. i dop. Moscow: GEOTAR-Media; 2. 2010 p.]

Alekberova Z.S. Behcet’s disease: monograph / Z. S. Alekberova. – M .: 2007. 86 p.

Singer O. Cogan and Behcet Syndromes. Rheum Dis Clin N Am 41 (2015) 75–91

Zeidan MJ, Saadoun D., Garrido M., Klatzmann D., Six A., Cacoub P. Beh? Et’s disease physiopathology: a contemporary review. Autoimmun Highlights, 2016 Dec; 7 (1): 4. DOI 10.1007 / s13317-016-0074-1

International Society for Beh? Et Disease. Official site:

International Study Group for Beh? Et’s Disease. Criteria for diagnosis of Beh? Et’s disease. Lancet 1990; 335: 1078-80. doi:

International Team for the Revision of the International Criteria for Beh? Et’s Disease (ITR-ICBD). The International Criteria for Beh? Et’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. Journal of the European Academy of Dermatology and Venereology, 2014, vol. 28, no. 3, pp. 338–347.

Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS, Houman H, Mahr A, Salvarani C, Sfikakis PP, Siva A, Al-Araji A. Diagnosis and management of Neuro-Beh? et “s disease: international consensus recommendations. J Neurol. 2014 Sep; 261 (9): 1662-76. doi: 10.1007 / s00415-013-7209-3.

Ovcharov P.S., Lisitsyna T.A., Veltishchev D. Yu., Tardov M.V., Burenchev D.V., Alekberova Z. S., Nasonov E. L. Neurological and mental disorders in Behcet’s disease. Journal of Neurology and Psychiatry. S.S. Korsakova. 2016; 116 (6): 76-86

Bhakta BB, Brennan P, James TE, Chamberlain MA, Noble BA, Silman AJ Beh? Et’s disease: evaluation of a new instrument to measure clinical activity. Rheumatology (Oxford). 1999; 38: 728–33.

Zouboulis C., Vaiopoulos G., Macromichelakis N. et al. Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades – Behcet’s disease in Greece. Clin. Exp. Rheum. 2003, 21, (suppl.30) S19 – S26

Leonardo NM, McNeil J. Behcet’s Disease: Is There Geographical Variation? A Review Far from the Silk Road. International Journal of Rheumatology. Volume 2015, Article ID 945262, 7 pages

Skef W, Hamilton MJ, Arayssi Th. Gastrointestinal Beh? Et »s disease: A review World J Gastroenterol 2015 April 7; 21 (13): 3801-3812. DOI: 10.3748 / wjg.v21.i13.3801

Hatemi G, Silman A, Bang D et al. Management of Beh? Et’s disease: a systematic literature review for the EULAR evidence based recommendations for the management of Behcet’s disease. Ann Rheum Dis 2008; 67: 1656–1662. doi: 10.1136 / ard.2008.087957.

Levy-Clarke G, Jabs DA, Read RW, et al. Expert panel recommendations for the use of antitumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology 2014; 121: 785–796.

Ozguler Y, Hatemi G. Management of Beh? Et’s syndrome. Curr Opin Rheumatol. 2016 Jan; 28 (1): 45-50. doi: 10.1097 / BOR.0000000000000231.

Noel N, Wechsler B, Nizard J, Costedoat-Chalumeau N, Boutin du LT, Dommergues M, Vauthier-Brouzes D, Cacoub P, Saadoun D. Beh? Et ‘s disease and pregnancy. See comment in PubMed Commons belowArthritis Rheum. 2013 Sep; 65 (9): 2450-6. doi: 10.1002 / art.38052.

Ben-Chetrit E. Beh? Et »s syndrome and pregnancy: course of the disease and pregnancy outcome. See comment in PubMed Commons belowClin Exp Rheumatol. 2014 Jul-Aug; 32 (4 Suppl 84): S93-8.

Hamza M., Ellench M., Zribi A. Behcet’s disease and pregnancy. Ann. Rheum. Dis. 1988, 47: 350

Jadaon J, Shushan A, Ezra Y, Sela HY, Ozcan C, Rojansky N. Beh? Et »s disease and pregnancy. See comment in PubMed Commons belowActa Obstet Gynecol Scand. 2005 Oct; 84 (10): 939-44.

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clinical picture

“I am 38 years old. Two years ago, for no reason, mouth ulcers began to appear. Having stopped eating, drinking, because chewing food is accompanied by wild pain, I lost 6-7 kilograms. In addition, vision deteriorated greatly.

Eyes constantly watery, sore, especially in daylight. In our district clinic, I went around almost all the doctors, but my diagnosis remains a mystery to everyone. Maybe your doctors will be able to declassify my illness and suggest treatment? ”

Most likely, our reader has Behcet’s disease (abbreviated as BB). Since 2012, this rare autoimmune disease belongs to the group of systemic vasculitis. Most often, an ailment occurs at the age of 20-40 years, and gives preference to men: they get sick 3-5 times more often than women.

Target Audience for these clinical guidelines

  • Rheumatologists;
  • general practitioners;
  • general practitioners;
  • ophthalmologists;
  • neurologists;
  • psychiatrists;
  • dentists;
  • gastroenterologists;
  • coloproctologists;
  • dermatovenerologists;
  • obstetrician-gynecologists;
  • cardiovascular surgeons.

search in electronic databases.

evidence base for recommendations are publications included in the Cochrane Library, EMBASE and MEDLINE databases.

Methods used to assess the quality and strength of evidence:

  • Expert consensus;
  • Reliability assessment in accordance with the levels (table 5).

Reliability is conditionally divided into four levels: A, B, C and D.

Table 5. Evidence Levels

Based on the findings of systematic reviews. A systematic review is obtained by a systematic search for data from all published clinical trials, a critical assessment of their quality and a generalization of the results by meta-analysis

Based on the results of at least several independent randomized controlled clinical trials

Based on the results of at least one clinical trial that does not meet quality criteria, for example, without randomization

The statement is based on expert opinion (expert consensus); no clinical studies

  • Assessment of the strength of the recommendations (indicated as level 1, 2 or “no gradation”) was made in accordance with the clinical recommendations of KDIGO (table 6)

Table 6. Strength assessment recommendations

on the part of patients

on the part of the doctor

Level 1 “Experts Recommend”

The vast majority of patients who find themselves in a similar situation would prefer to follow the recommended route, and only a small fraction of them would reject this route

The vast majority of their patients will be advised by the doctor to follow this particular path.

The recommendation can be accepted as a standard for medical personnel in most clinical situations.

Most patients who find themselves in a similar situation would have favored following the recommended path, but a significant proportion would have rejected this path.

For different patients should choose different options for recommendations that are suitable for them. Each patient needs help in choosing and making a decision that will correspond to the values ​​and preferences of this patient

Recommendations are likely to require discussion with all stakeholders before being accepted as a clinical standard.

This level is used in cases where the common sense of the expert researcher is based on the recommendation or when the topic under discussion does not allow adequate application of the evidence system used in clinical practice.

Methods used to analyze evidence:

  • Reviews of published meta-analyzes;
  • Systematic reviews with tables of evidence.

Recommended good practice is based on the clinical experience of members of the working group to develop recommendations.

Cost analysis was not carried out and publications on pharmacoeconomics were not analyzed.

Recommendation validation method:

  • External peer review;
  • Internal peer review.

These recommendations in a preliminary version are reviewed by independent experts.

Behcet’s syndrome is in the nosological sense a purely clinical concept. Not only relatively specific, but even any characteristic histomorphological, immunological or biochemical signs of this disease do not exist. At the same time, the clinical picture with a sufficiently complete severity makes the diagnosis of the disease indisputable. It should be borne in mind that specific symptoms of Behcet’s syndrome may not occur simultaneously.

Months and even years may elapse between the onset of the first signs of the disease and the addition of new clinical manifestations. By this time, the first signs may undergo a complete reverse development. Therefore, in each specific period, a patient with Behcet’s syndrome can have only one or several characteristic signs of the disease, not to mention the possibility of remission without any pathological symptoms. This circumstance gives particular importance to anamnestic data for the correct recognition of the disease.

The most common clinical manifestations of Behcet’s syndrome are described below.

Terms and definitions

Glucocorticoids (HA) are synthetic steroid hormones with natural anti-inflammatory activity.

Basic anti-inflammatory drugs (BPVPs) – immunosuppressive drugs that have anti-inflammatory and disease-modifying effects, are divided into synthetic (s-BPVP), synthetic synthetic (t-BPVP) and biological (b-BPVP)

Genetically engineered biological drugs (GIBP) is a synonym for biological BPVP adopted in Russia. A group of drugs characterized by a selective effect on certain mechanisms of the development of chronic inflammation and representing monoclonal antibodies to immunocompetent cells or pro-inflammatory cytokines, hybrid protein molecules that inhibit the activity of cytokines and the interaction of immunocompetent cells

Inhibitors of tumor necrosis factor-alpha (i-TNF-α) are artificially synthesized monoclonal antibodies and hybrid proteins that block the activity of the pro-inflammatory cytokine TNF [1].

Behcet’s disease (BB) is a systemic vasculitis of unknown etiology, characterized by relapses of the ulcerative process in the oral cavity and genitals, damage to the eyes, joints, gastrointestinal tract (GIT), central nervous system (CNS) and other organs. It has a chronic course with unpredictable exacerbations and remissions [2].

Aphthous (ulcerative) stomatitis

Shallow painful ulcers on the oral mucosa are usually the first symptom of the disease and occur in almost all patients. These ulcers (diameter from 2 to 10 mm) are located in the form of single elements or clusters. They are localized on the mucous membrane of the cheeks, gums, tongue, lips, sometimes in the pharyngeal region, less often in the larynx and on the nasal mucosa.

Appendix B. Patient Information

Information about the effect of pregnancy on BB and vice versa is scarce and, in many ways, contradictory. There are indications that in some cases, pregnancy leads to an exacerbation of BB [17], in others, on the contrary, an improvement in the symptoms of the disease during pregnancy has been observed [17, 18]. It was noted that the risk of complications of pregnancy and childbirth is higher in women with a history of BB and thrombosis [17].

Given the chronic course of BB, patients should be treated continuously, in most cases, for life. In order to achieve the best treatment result and achieve remission of the disease, correction and cancellation of drugs prescribed by the doctor are not allowed independent, not agreed with the attending physician. A visit to the doctor should be made with an exacerbation of BB as soon as possible, as well as in a planned manner to assess the effectiveness and tolerability of therapy every 1-3 months in the first year after exacerbation and then every 6-12 months in the absence of exacerbation.

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Genital ulcers

Multiple or single recurrent painful genital ulcers look very much like oral ulcers. They are found in approximately 80% of patients with Behcet’s syndrome and are localized in women on the labia, vulva and vaginal mucosa, and in men on the scrotum and rarely on the penis.

Both women and men can have perineal skin lesions. Vaginal mucosa ulcers are often painless and in such cases they are detected either in connection with complaints of discharge, or during gynecological examination for other reasons. In most patients, recurrence of genital ulcers is less common than oral; the course and terms of healing do not differ.

Ulcers of the mucous membrane of the bladder or symptoms of cystitis without signs of ulceration are rarely observed.

Vascular injury

The most serious and frequent eye lesion is uveitis, which occurs in 2/3 of patients. As a rule, it is bilateral and very rarely occurs at the very beginning of the disease. Between the first manifestations of the disease (usually an ulcerative lesion of the mucous membrane of the mouth and genitals) and the onset of uveitis, on average, about XNUMX years pass.

A patient with or with suspicion of Behcet’s syndrome should be consulted by an ophthalmologist with an examination of the anterior chamber of the eye through biomicroscopy and the posterior segment using biomicroscopic and indirect ophthalmoscopy. Photographs of the fundus after intravenous administration of fluoroscein often reveal diffuse staining of the disc and retina, which indicates a significantly increased vascular permeability.

Precipitates in the cornea and cellular infiltrates in the vitreous body definitely indicate the activity of uveitis.

It is now apparent that the main cause of blindness in Behcet’s syndrome is posterior uveitis with involvement of the vitreous body and retina. Blindness can also cause anterior uveitis with the development of hypopion (i.e., accumulation of pus in the anterior chamber of the eye). However, at present, hypopion is rare in patients with Behcet’s syndrome, which is associated with local or systemic use of corticosteroids.

Another ocular pathology in this disease includes conjunctivitis, ulceration of the cornea and less commonly observed optic neuritis, chorioretinitis, retinal vasculitis, arterial thrombosis or fundus veins. Eye lesions in some cases are completely reversible, but a number of patients develop persistent severe changes, in particular, in addition to partial or complete loss of vision, glaucoma and cataracts may occur.

Arthritis develops in approximately 50% of patients with Behcet’s syndrome in any period of the disease. Usually they are combined with other manifestations of the disease, very often with erythema nodosum. As a rule, there is a symmetric polyarthritis affecting large joints (usually knee, ankle, elbow). Less commonly observed is monoarthritis of a large joint or involvement of small joints in the process. Typically, the joints are affected by the type of acute often recurring synovitis with complete reverse development.

When analyzing synovial fluid, from 5000 to 20 leukocytes in 000 mm1 are detected with a predominance of neutrophils. The chronic course of arthritis is very rare (in this case, only a few mononuclear leukocytes can be contained in the synovial fluid), and even minimal signs of articular destruction are an exception (including with frequent relapse of arthritis for many years). Arthralgia without objective signs of joint inflammation is very common, but does not have diagnostic value.

In single patients who are carriers of HLA-B27, the development of sacroileitis and spondylitis is described.

Skin changes are characteristic of approximately 70% of patients with Behcet’s syndrome and are considered as manifold manifestations of cutaneous vasculitis. These include erythematous papules, pustules, vesicles, and elements such as erythema nodosum. The latter may not differ in any way from the “usual” erythema nodosum, but in some cases demonstrate some features.

The formation of a small pustule or nodule (sometimes with a pink corolla) a day after any injection at the injection site is almost pathognomonic for Behcet’s syndrome, although it is by no means found in all patients. This feature can also be used for diagnostic purposes – after the administration of an isotonic sodium chloride solution or even an injection with a dry needle, a sterile pustule sometimes appears.

In addition to the manifestations of cutaneous vasculitis discussed above, thrombophlebitis of superficial or deep veins often develops. More often it is localized on the legs and sometimes is the cause of pulmonary embolism. Very rarely observed thrombosis of the superior or inferior vena cava. Sometimes phlebitis develops immediately after venipuncture.

The involvement of arteries in the pathological process is rare and is manifested by thrombosis and the formation of aneurysms. These changes were found in the aorta, ulnar, femoral and popliteal arteries, and fundus vessels. Pulmonary thrombosis was also described. Thrombosis of the arteries of the legs can lead to gangrene of the feet.

A number of patients with Behcet’s syndrome are characterized by diarrhea, nausea, symptoms of proctitis, perianal ulcers. Sometimes intestinal lesions are indistinguishable from ulcerative colitis and Crohn’s disease. Some authors emphasize the pathogenetic proximity of these diseases and indicate that in the case of primary Crohn’s disease, all signs of Behcet’s syndrome may occur.

In some patients with a combination of symptoms of Behcet’s syndrome and Crohn’s disease, resection of the affected part of the colon also led to a marked reduction in symptoms of Behcet’s syndrome. In single patients, duodenal ulcers were detected. Gastrointestinal manifestations are especially frequent in the Japanese (50% of all patients).

1.3 Epidemiology

The disease is most common in countries that are located along the historic Great Silk Road, stretching from East Asia to the Mediterranean. More rarely, BB is found in North and South America, as well as in Northern Europe. Epidemiological studies show the highest prevalence of the disease in Turkey (80-370 per 100 thousand.

), Iran and Iraq (70 per 100 thousand), China (14 per 100 thousand), Japan (11,9 people per 100 thousand population). In Europe, the average incidence of the disease is 2,5 per 100 thousand people [2, 5]. There are no epidemiological data on the prevalence of BB in Russia. It is known that BB is most often found in Russia among natives of the North Caucasus.

BB is 2-3 times more common among men in Turkey and the Arab countries, but in Japan and some European countries women are sick more often than men. Most often, BB makes its debut between the ages of 20 and 39, both among men and women.

The prognosis for BB is most often favorable, but about 25% of patients with eye damage in the absence of adequate therapy have a significant decrease in visual acuity.

Mortality in BB is 9% and, as a rule, is due to the involvement of the central nervous system and large vessels. Risk factors for a more severe course of the disease are the age of development of the disease up to 25 years and the male gender [1, 2].

Neurological pathology

Significant neurological disorder is established in the presence of the following three criteria:

  1. Patient meets international BB criteria (ISGBD *)
  2. Identified neurological syndromes (with objective neurological manifestations) should be caused by BB and should be confirmed by characteristic changes in one or both of the following examination methods:

b) cerebrospinal fluid (CSF)

  1. There should be no other reason for the revealed neurological manifestations, except for BB

A probable neurological disorder is established in the presence of one of the following criteria and in the absence of other reasons for the identified neurological disorders:

  1. Neurological syndrome is characteristic of a reliable neurological lesion, combined with systemic manifestations of BB, but the patient does not meet the international criteria for BB (ISGBD)
  2. An uncharacteristic neurological syndrome encountered in the context of international BB criteria (ISGBD)

Note: * ISGBD – International Study Group Behcet Disease Criteria – international criteria for the diagnosis of BB 1990 or any other criteria that are currently accepted or in the future

A specific marker of the disease does not exist, it is determined by the combination of symptoms. A rheumatologist can diagnose the syndrome, who, if necessary, will connect specialists from related professions to the treatment process. The doctor will analyze the symptoms and collect a family history. If you suspect the presence of Behcet’s disease, treatment is not prescribed until a patergic test is performed, which will confirm the diagnosis. In patients with pathology, after a day (sometimes two), a rash of 3-10 mm in size appears at the injection site.

Laboratory studies also occupy an equally important place in the diagnosis of Behcet’s disease:

  • immunological tests;
  • general and biochemical blood test;
  • coagulogram;
  • joint radiography
  • study of synovial fluid.

– in severe forms of the disease in almost 1/4 of patients. The main neurological pathology in Behcet’s syndrome is meningoencephalitis, which usually does not occur in the early stages of the disease and manifests itself in fever, severe headache, and stiff neck. The cerebrospinal fluid is sterile and is characterized by lymphocytosis and a normal content of gamma globulins. Meningoencephalitis, which can occur with exacerbations and remissions, makes the prognosis of the disease much more serious.

Other types of damage to the nervous system are less common – stem and spinal disorders, impaired corticospinal conduction, reversible increase in intracranial pressure with edema of the optic disc, transient pathology of the cranial nerves. Peripheral neuropathies are rare. In some patients hemiparesis, tetraplegia, “benign” increase in intracranial pressure are described. Psychoses are very rare.

1.5 Classification

I. Complete type: in the presence of 4 large signs in a patient

II. Incomplete type:

    • 3 great signs
    • 2 large and 2 small signs
    • typical eye symptoms combined with 1 large or 2 small symptoms
    • only 2 big signs
    • 1 large and 2 small signs

Classification signs of BB are presented in Appendix G2.

Table 1. Classification of neurological disorders in Behcet’s disease [8, 9]

Damage to the central nervous system

  • Multifocal / diffuse
  • Brain damage
  • Spinal cord injury
  • Cerebellar lesion
  • Asymptomatic (mute) lesion
  • Optic neuropathy
  • Thrombosis of the venous sinuses of the brain: intracranial hypertension
  • Aneurysms of the arteries of the brain
  • Aneurysms / stratification of extracranial arteries
  • Acute meningeal syndrome

Damage to the peripheral nervous system (connection with BB is not certain)

  • Peripheral Neuropathy or Multiple Mononeuritis
  • Myopathy or myositis

Mixed parenchymal and non-parenchymal lesions

  • To assess the total activity of BB, it is recommended to use the BDCAF index (Beh? Et Disease Current Activity Form), proposed by the International Society for Beh? Et Disease (ISBD)) [5, 10] (see Appendix D1)
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The level of evidence reliability is A, the level of credibility of the recommendation is 1

  • To determine the severity of BB, it is recommended to use the classification proposed by Ch. Zouboulis et al. [eleven]

The level of reliability of evidence is A, the level of credibility of the recommendation is 1

Low severity implies the presence of patients:

  • aphthae of the oral mucosa
  • genital ulcers
  • typical skin lesions (erythema nodosum, papulopustular rashes, folliculitis, leukocytoclastic vasculitis)
  • arthralgia
  • recurrent headaches
  • epididymitis
  • minor gastrointestinal symptoms (chronic diarrhea, recurring abdominal pain)
  • pleural chest pain
  • superficial venous thrombosis.

The average severity is determined in those patients whose clinical picture includes:

  • arthritis
  • deep vein thrombosis of the lower extremities
  • front uveitis
  • gastrointestinal bleeding.

Тяжелая форма ББ включает:

  • задний увеит, панувеит, васкулит сетчатки
  • тромбоз артерий или аневризмы
  • тромбоз крупных вен (нижняя полая вена, печеночная вена)
  • неврологические проявления (гемипарез, психоэмоциональные расстройства и др)
  • перфорацию кишечника.

2.1. Жалобы и анамнез

  • При сборе анамнеза для диагностики ББ рекомендовано учитывать следующее:
    • Наличие повторных случаев заболевания в семьях больных (семейная агрегация);
    • Эпизоды афтозного стоматита (часто с детского возраста);
    • Эпизоды внезапного ухудшения зрения, или «туман» перед глазами;
    • Обращения к урологу по поводу отека мошонки;
    • Расстройства стула (диарея с кровью);
    • Эпизоды тромбофлебита в анамнезе, преимущественно вен нижних конечностей (чаще у лиц мужского пола);
    • Наличие в анамнезе каких-либо церебральных симптомов, например, эпилептиформных приступов.

Уровень достоверности доказательств — А, уровень убедительности рекомендаций – 1

Другие проявления болезни

Среди более редких симптомов синдрома Бехчета следует указать на возможность вовлечения в процесс легких с их диффузной инфильтрацией, кровохарканьем и плевритом (почти исключительно у турецкого населения). Известны также поражения сердца (особенно при тяжелых генерализованных формах болезни): миокардит, перикардит, эндокардит с формированием умеренно выраженных клапанных пороков.

Нечастое осложнение синдрома Бехчета — системный вторичный амилоидоз, особенно среди жителей Ближнего Востока.

Отдельные авторы обращали внимание на предположительно неслучайные сочетания синдрома Бехчета с лимфобластной лимфомой и с сопутствующей инфекцией (в частности, подногтевой), что может косвенно указывать на свойственные заболеванию иммунные нарушения.

Если Вас интересуют еще какие-нибудь виды болезней и группы заболеваний человека или у Вас есть какие-либо другие вопросы и предложения –

, мы обязательно постараемся Вам помочь.

Лабораторные показатели

В активную фазу болезни обычно отмечают увеличение СОЭ, нерезкий нейтрофильный лейкоцитоз, умеренную анемию, повышение уровня С-реактивного белка, фибриногена и a

-глобулинов. Часто обнаруживается поликлональная гипергаммаглобулинемия и повышение уровня отдельных классов иммуноглобулинов.

При обострениях синдрома Бехчета отмечают нарастание уровня иммунных комплексов, 9-го компонента комплемента и хемотаксической активности нейтрофилов, образование розеткообразных скоплений тромбоцитов вокруг нейтрофилов, появление криоглобулинов и антител к клеткам слизистых оболочек, снижение фибринолиза, ревматоидный фактор, ядерные антитела и LE-клетки не определяются.

Диагноз

Для диагноза синдрома Бехчета считается достаточным сочетание язвенного (афтозного) стоматита, язвенного поражения половых органов и воспаления тканей глаза (прежде всего увеита). Очень большое значение имеют также такие признаки, как артрит, менингоэнцефалит и рассмотренные выше кожные изменения. J. O’Duffy (1981) предлагает считать диагноз достоверным при наличии трех из следующих б клинических признаков: 1) рецидивирующий афтозный стоматит; 2) афтозные язвы половых органов; 3) увеит; 4) синовит; 5) кожный васкулит; 6) менингоэнцефалит.

Наличие афтозного стоматита является обязательным симптомом. Если афтозные язвы полости рта или половых органов сочетаются только с одним из перечисленных клинических признаков, то с некоторым основанием может быть диагностирована «неполная» форма синдрома Бехчета. Важно иметь в виду, что все рассмотренные симптомы могут развиваться неодновременно.

Необходимо помнить о таком фактически специфическом (хотя и совершенно необязательном) симптоме, как появление кожных пустул после любых инъекций. Несомненное значение имеют и отмеченные выше определенные национальные особенности. В бывшем СССР заболевание встречается обычно среди коренных жителей Кавказа и Закавказья.

В единичных случаях возникает вопрос о дифференцировании синдромов Бехчета и Рейтера, которым свойственны такие общие черты, как поражение кожи и слизистых оболочек,. острый несимметричный полиартрит, воспаление тканей глаза, иногда также неврологическая патология. Однако при синдроме Рейтера стоматит бывает очень редко, язвы половых органов и слизистой оболочки рта обычно безболезненны, глазная патология характеризуется, как правило, конъюнктивитом (а не увеитом), поражение нервной системы является скорее исключением и не отличается особой тяжестью. Кроме того, синдрому Рейтера совершенно несвойственны тромбофлебит и узловатая эритема.

В то же время явная клиническая картина спондилита в гораздо меньшей степени присуща синдрому Бехчета. Отдельные больные с язвенным колитом могут иметь клиническую картину, практически неотличимую от синдрома Бехчета с симптомами колита (рецидивирующие афтозные язвы слизистой оболочки рта, артрит, узловатая эритема, ирит).

Однако при язвенном колите не развиваются язвы половых органов и задний увеит, а афты полости рта обычно не бывают тяжелыми. Кроме того, при колоноскопии удается установить, что у больных с синдромом Бехчета и наличием язв в толстой кишке эти язвы (в отличие от язв при собственно язвенном колите) окружены внешне нормальной, а не воспаленной слизистой оболочкой.

В дифференциальной диагностике нужно также иметь в виду синдром Стивенса—Джонсона, который развивается чаще у мужчин до 20 лет, причем особенно часто в детском возрасте. Заболевание начинается, как правило, остро, с высокой температурой и быстрым последующим развитием тяжелого язвенного стоматита. Язвенные изменения могут возникнуть также на слизистой оболочке носа, конъюнктиве, половых органах и (что важно для диагноза) вокруг ануса.

В отличие от синдрома Бехчета при синдроме Стивенса—Джонсона не бывает артрита (хотя артралгии и миалгии нередки), увеита, тромбофлебита, классической узловатой эритемы, неврологических проявлений, колита, эпидидимита и таких сосудистых осложнений, как аневризмы, периферическая гангрена, кровоизлияния в ткани глаза.

Естественно, что у больных с банальным рецидивирующим афтозным стоматитом, в том числе с повышенной температурой тела, синдром Бехчета (как и синдром Стивенса—Джонсона) исключается благодаря отсутствию других характерных клинических признаков. Кроме того, при банальном язвенном стоматите не обнаруживают таких характерных для синдрома Бехчета лабораторных признаков, как нарастание уровня С-реактивного белка, фибриногена и a2-глобулинов, высокая СОЭ, гипергаммаглобулинемия.

2.4. Инструментальная диагностика

  • Рекомендовано при подозрении на поражение глаз у пациента с ББ обследование у офтальмолога с биомикроскопией (исследованием с помощью щелевой лампы) и офтальмоскопией (исследованием глазного дна)
  • рекомендовано при подозрении на тромбоз периферических сосудов у пациента с ББ проведение ультразвуковой допплерографии данных сосудов
  • Рекомендовано проведение КТ или МРТ с контрастированием при подозрении на тромбоз сосудов легких или брюшной полости у пациента с ББ
  • Рекомендовано выполнение эзофагогастродуоденоскопии/ колоноскопии при подозрении на поражение ЖКТ в рамках ББ
  • Биопсия нервной ткани иногда может быть полезной для диагностики неврологических расстройств при ББ, но она не рекомендуется как часть диагностического процесса. [8, 9]

Уровень достоверности доказательств — D, уровень убедительности рекомендаций – 2

Прогноз и профилактические меры

Прогнозы болезни Бехчета зависят от органов и систем, которые вовлечены в патологический процесс. При изолированной форме заболевания, когда поражены только кожные покровы и слизистые оболочки, прогноз, как правило, благоприятный.

В случае развития увеита качество жизни пациента может быть ухудшено частичной или полной слепотой, а наиболее опасными являются ситуации поражения ЦНС и сосудов – в таких случаях примерно 5% больных умирают от развившихся осложнений. По причине невыясненной этиологии заболевания меры его профилактики пока не разработаны.

Синдром Бехчета относится к редким, но достаточно сложным заболеваниям, а качество жизни пациентов во многом зависит от их внимательного отношения к собственному здоровью.

Поэтому при возникновении каких-либо тревожных признаков нужно не только сразу же обратиться к врачу и пройти соответствующие исследования, но и обязательно следить за своим состоянием, чтобы не допустить присоединения новых симптомов и развития осложнений.

Течение синдрома Бехчета весьма вариабельно. В разные периоды болезни число клинических симптомов и их выраженность различны. Заболеванию свойственны рецидивы и периоды затихания активности. Практически при каждом обострении имеют место повышение температуры тела и афтозный стоматит, очень часто в сочетании с кожными изменениями. Ремиссии продолжаются от нескольких недель до нескольких лет, что во многом определяет общую тяжесть болезни и трудоспособность больного.

In some patients, Behcet’s syndrome is easy and does not significantly affect their life. On the other hand, in some cases, the disease is very difficult, with high fever, obvious intoxication, polysystemic lesions and the absence of complete remissions. The causes of death are meningoencephalitis and vascular lesions of the central nervous system (most often), arterial thrombosis of various locations, embolism due to thrombophlebitis, perforation of ulcers of the digestive tract. The development of such signs significantly worsens the prognosis of the disease.

Severe uveitis that does not affect life expectancy may nevertheless indicate a difficult prognosis due to the threat of loss of vision.

In the presence of an isolated form of the disease, when the pathological process affects only the mucous membranes and the skin, the prognosis is favorable. If the disease has affected the organs of vision, complete or partial blindness may occur due to atrophy of the optic nerve. Of particular danger are painful changes in the central nervous system and blood vessels of various calibers. In this case, about 5% of patients have a fatal outcome.

Since the causes of the syndrome are unknown, no particular prophylaxis against it exists. Since the disease refers to chronic diseases, the main preventive measures are reduced to prolonging remission and preventing exacerbations. To do this, it is recommended to lead a healthy lifestyle, do not stop taking the medications prescribed by your doctor, inform the rheumatologist in time about the appearance of new symptoms, and undergo a regular examination.

2.5. Differential diagnosis

  • It is recommended to differentiate BB with the following diseases and conditions: herpetic and HIV infections, pemphigus, keratoderma, Sweet syndrome, reactive arthritis, Crohn’s disease, ulcerative colitis, sarcoidosis, erythema nodosum (associated with other diseases), hematological diseases, multiple sclerosis, infectious meningoencephlomyelitis.
  • It is recommended that neurological disorders in BB be included in the differential diagnosis of multiple sclerosis, acute cerebrovascular accident in young people, as well as in patients with intracranial hypertension, meningoencephalitis and myelitis.

Forecasts and Prevention

Primary prevention of BB is not developed. Patients should be advised to avoid factors that can provoke an exacerbation of the disease (intercurrent infections, stress, trauma, etc.), to quit smoking.

Reliability level of evidence D, level of credibility of recommendation 1

Screening measures are recommended for the early diagnosis of BB among people with recurrent aphthous stomatitis who are at risk of developing this disease (first-degree relatives or others have BB).

Clinical observation of patients with BB is recommended.

Preventive measures aimed at preventing the development of Behcet’s disease do not exist. But many experts recommend adhering to prevention, which is aimed at maintaining a normal level of immune defense of the body. Such a preventative complex includes:

  • Compliance with the mode of the day.
  • Compliance with a balanced and proper diet.
  • Quitting alcohol and smoking.
  • Daily feasible physical activity.
  • Decrease in psychoemotional load.
  • Timely treatment of infectious diseases.
  • Strict implementation of all medical recommendations.
  • The exception to taking self-prescribed drugs.

Behcet’s disease in women is detected very rarely, since the pathology has a symptomatology similar to many diseases. If untreated, this disease in a short time can lead to the development of complete blindness and even death. Therefore, you must carefully monitor changes in your body and, if necessary, urgently seek qualified medical help.

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Svetlana Borszavich

General practitioner, cardiologist, with active work in therapy, gastroenterology, cardiology, rheumatology, immunology with allergology.
Fluent in general clinical methods for the diagnosis and treatment of heart disease, as well as electrocardiography, echocardiography, monitoring of cholera on an ECG and daily monitoring of blood pressure.
The treatment complex developed by the author significantly helps with cerebrovascular injuries and metabolic disorders in the brain and vascular diseases: hypertension and complications caused by diabetes.
The author is a member of the European Society of Therapists, a regular participant in scientific conferences and congresses in the field of cardiology and general medicine. She has repeatedly participated in a research program at a private university in Japan in the field of reconstructive medicine.

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