[Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity].

Uncontrolled hypercoagulation and inflammation (thromboinflammation), which are both independent and closely related and amplifying each other pathological processes, form the basis for pathogenesis of a wide range of diseases and complications, including immuno-inflammatory (autoimmune) rheumatic diseases, with the development of potentially fatal injuries of internal organs. Thrombotic microangiopathy is one of the most prominent prototypes of thromboinflammatory pathological conditions. The close link between environmental factors, hemostasis genetic defects and the complement system, inflammation and autoimmunity as pathogenetic mechanisms of microthrombosis draws particular attention to studying thrombotic microangiopathy in immuno-inflammatory rheumatic diseases, primarily systemic lupus erythematosus, antiphospholipid syndrome and scleroderma renal crisis. In future, these studies may be important for expanding the idea of the role of autoimmune mechanisms in pathogenesis of critical hemostasis disorders in human diseases, and for developing new approaches to therapy. Recently, special attention has been paid to the treatment of systemic lupus erythematosus and antiphospholipid syndrome with eculizumab, which is humanized monoclonal IgG2/4k antibody that blocks the complement component C5a and the membrane attack complex (C5b-9) formation, and which is registered for the treatment of atypical hemolytic uremic syndrome, paroxysmal nocturnal hemoglobinuria, as well as severe forms of myasthenia gravis and neuromyelitis optica. Further studies in this direction will create prerequisites for improving the prognosis not only in patients with orphan disorders, but also for widespread human diseases.

[Intestinal manifestations of Behçet's disease].

Behçet's disease (BD) is a systemic vasculitis of unknown origin, characterized by recurrences of the ulcerative process in the oral cavity and on the genitals, inflammatory damage of the eyes, joints, vessels and other organs. The severity and prognosis of BD determines organ pathology. Intestinal manifestations of BD (intestinal BD) are the least studied. Its verification in BD is complicated by the variety of clinical manifestations, their similarity with inflammatory bowel diseases, the lack of informative laboratory tests, pathognomonic endoscopic and histological signs. Intestinal BD can lead to serious complications (massive bleeding, intestinal perforation and fistula formation), which can not only significantly reduce the quality of the patient's life, but also cause death. Treatment of intestinal BD is not standardized; it is mainly empirical and conducted courses. The purpose of therapy is to achieve clinical remission, healing of intestinal ulcers and prevention of surgery. The article presents a case of severe refractory intestinal BD, requiring twice emergency surgical care - removal of half and then the whole of the colon because of multiple perforations. A brief review of the literature is given and diagnostic difficulties of intestinal BD are discussed.

Comparative analysis of anxiety-depressive spectrum disorders in patients with rheumatic diseases.

AIM: Research objective - comparative analysis of incidence and structure of anxiety-depressive spectrum disorders (ADD) in patients with various rheumatic diseases (RD). MATERIALS AND METHODS: 613 patients with RD were enrolled in the study: 180 with a reliable diagnosis of systemic lupus erythematosus (SLE), 128 with rheumatoid arthritis (RA), 110 with systemic sclerosis (SSc), 115 with Behcet's disease (BD), 80 with primary Sjögren's syndrome (pSS). Female prevailed in all groups (95% of patients with pSS, 88,2% - SSc, 87,2% - RA, 85,5% of SLE) except BD patients (70% male). The mean age was 42.3±1.54 years and was lower in patients with BD (33.3±0.98 years) and SLE (34.6±0.93 years) compared to patients with SSc (49.9±2.47 years), RA (47.4±0.99 years) and pSS (46.2±2.3 years). The mean RD duration was 130,0±8,65 months and was more at BD - 148,5±10,4 months, pSS - 141,6±8,92 months, RA - 138,4±10,1months, and less at SLE - 134,9±8,8 months and SSc - 87,0±5,04 months. The mean SLE activity index SLEDAI was 9,13±0,63 points (high), RA (DAS28) - 5,26±0,17 points (high), BD (BDCAF) - 3,79±0,2 points (moderate) and SSc by G. Valentini - 1,1±0,20 points (moderate). Glucocorticoids took 100% of patients with pSS, 91,1% - SLE, 90% - SSc, 87% - BD and 67,2% - RA patients; conventional disease modifying anti-rheumatic drugs (cDMARDs) took 90% of patients with SSc, 84% - BD, 79,6% - RA, 68% - pSS, 40,6% - SLE. Biologic DMARDs took 32% of patients with RA, 17,4% - BD, 7,3% - SSc and 7,2% - SLE. Mental disorders were diagnosed by psychiatrist as a result of screening by the hospital anxiety and depression scale (HADS) and in semi-structured interview in accordance with the ICD-10/ DSM-IV. The severity of depression was evaluated by Montgomery-Asberg Depression Rating Scale (MADRS) and anxiety - by Hamilton Anxiety Rating Scale (HAM-A). Projective psychological methods were used for cognitive impairment detection. RESULTS: Screening of depressive disorders (HADS-D≥8) was positive in 180 (29,4%) patients with RD, including 74 (41%) patients with SLE, 38 (35%) - SSc, 29 (23%) - RA, 23 (20%) - BD and 16 (20%) - pSS; anxiety disorders (HADS-A≥8) - in 272 (44,4%) patients, including 66 (52%) patients with RA, 40 (50%) - pSS, 77 (43%) - SLE, 45 (41%) - SSc and 44 (38%) - BD. In accordance with the ICD-10/ DSM-IV depressive disorders have been identified in 389 (63%) patients, including 94 (73%) patients with RA, 71 (64,5%) - SSc, 69 (60%) - BD, 90 (50%) - SLE and 39 (49%) - pSS; anxiety disorders - in 377 (61,5%) patients, including 20 (25%) patients with pSS, 44 (24,5%) - SLE, 29 (23%) - RA, 20 (17%) - BD and 7 (6,4%) - SSc. CONCLUSION: Anxiety-depressive spectrum disorders are typical for most patients with RA, SLE, SSc, pSS and BD. ADDs diagnosis in RD patients with the use of the HADS did not reveal a significant proportion. To obtain objective data on the frequency and structure of ADDs, psychopathological and clinical psychological diagnosis is necessary.

[Behçet's disease: Intracardiac thrombosis (a description of two cases and a review of literature)]. / Bolezn' Bekhcheta: vnutriserdechnyi tromboz (opisanie dvukh nabliudenii i obzor literatury).

Behçet's disease (BD) is systemic vasculitis of unknown etiology, which is more common in the countries located along the Great Silk Road. The disease is diagnosed if a patient has 4 key diagnostic signs: aphthous stomatitis, genital sores, and eye and skin lesions. Vascular diseases referred to as minor criteria for BD are characterized by the formation of aneurysms and thrombosis, predominantly in the venous bed. In venous disorders, a blood clot can form in any vessel, including caval, cerebral, pulmonary, and other veins. The paper describes two clinical cases of BD with intracardiac thrombosis. In one case, a 24-year-old male patient with a documented diagnosis of BD, echocardiography revealed a left ventricular spontaneous echo contrast phenomenon that disappeared due to immunosuppressive therapy. The other case was a 34-year-old female patient, in whom the diagnosis was based on the international disease criteria: aphthous stomatitis, skin lesions (pseudopustulosis, erythema nodosum), and genital sores. Computed tomographic angiography showed a 3.7×2.2-cm mass (thrombus) in the right atrium. In addition, blood clots were present in the hepatic and inferior vena cava. No abnormalities in the coagulation system were found in both cases.

[Behçet's disease and malignization: literature review and authors' data].

The paper describes cancers (enteric adenocarcinoma in one case and acute leukemia in the other) in 2 patients with Behçet's disease (BD) followed up at the V.A. Nasonova Research Institute of Rheumatology. It gives the data available in the literature on BD concurrent with malignant tumors. This concurrence is noted to substantially worsen the prognosis of the disease as a whole.

[Behçet's disease: clinical and demographic associations].

AIM: To comparatively study the clinical manifestations, sexual and HLA-B51 associations in patients with Behçet's disease (BD) in two ethnic groups. SUBJECTS AND METHODS: The authors examined 143 patients with the valid diagnosis of BED who were divided into 2 groups: 1) 85 patients, the dwellers of Dagestan (a multiethnic cohort), 63 men and 22 women (mean age 29 +/- 7.4 years); 2) 58 Russian men and women (mean age 33 +/- 11.7 years). RESULTS: Two major criteria for BD, such as aphthous stomatitis and external genital ulcers, were found with the same frequency. Panuveitis and angiitis of the retina were diagnosed more frequently in the Dagestani population with BD than in the Russians. Out of the minor criteria for BD, the incidence of lower limb deep venous thrombosis was 23% for the Dagestanis versus 3% for the Russians. Arterial thromboses and pulmonary artery aneurysms became causes of death in 4 in 5 men aged 19-23 years from their Dagestani ancestry. HLA B51 (B marker) was found in the dwellers of Dagestan: in 70% of the men and 40% of the women who had BD. CONCLUSION: BD runs a more severe course in male patients and is characterized by severe eye diseases and the systematic pattern of the process at young age. Gender-specific and genetic aspects call for further comparative investigations on large ethnic patient cohorts of other ancestries.

[Behcet's disease and associations with HLA-B5 antigen (a review of literature and the authors' findings)].

AIM: To estimate the distribution of HLA Class I (A, B) antigens in patients with Behcet's disease (BD) and the association of HLA-B5 antigen with the clinical manifestations of the disease in different ethnic and population groups in relation to gender. SUBJECTS AND METHODS: The study covered 93 patients (68 males, 25 females) from the representatives of 24 ethnicities with the verified disease. HLA Class I antigens were typed by the microlymphocytotoxic technique, by applying an antileukocytic serum kit (GISANS, Saint Petersburg). RESULTS: In patients with BD, the prevalence of HLA-B5 antigen proved to be significantly higher than that in the controls (72.0 and 21%, respectively) and to be similar in patients of different ethnicities living in the Caucasus and Transcaucasus (80-83%) while the number of HLA-B5 antigen-positive patients with BD was thrice less in the Russian population than in other BD patients (p < 0.01). There was a significant correlation of HLA-B5 antigen with ocular lesion (retinal angiitis) predominantly in male patients with BD. CONCLUSION: The prevalence of HLA-B5 antigen was higher in patients with BD than in the population-based control. The diagnostic value of this antigen is not so great, for example, in the Russian population of patients with BD. The presence of HLA-B5 antigen in the phenotype of male patients with BD may be regarded as a prognostically poor marker of development of eye diseases.

[Cognitive disorders in Behçet's disease]. / Kognitivnye narusheniia pri bolezni Bekhcheta.

AIM: To study the prevalence of cognitive disorders (CD) and clinical/pathogenetic correlations of CD in patients with Behçet's Disease (BD). MATERIAL AND METHODS: One hundred and six BD patients were enrolled in the study. The majority of patients were natives of the North Caucasus (51.9%). Mean age was 33.3±0.98 years, mean illness duration 148.5±10.4 months. All the patients met the criteria of the International Study Group for BD (1990) classification. The disease activity was assessed by scoring system BDCAF. A diagnosis of a mental disorder (MD) was established by the psychiatrist in accordance with the ICD-10 using a semi-structured interview. The Montgomery-Asberg Depression Rating Scale (MADRS), the Hamilton Anxiety Rating Scale (HAM-A), a pathopsychological method 'Pictograms', clinical/psychological methods for assessment of cognitive functions (memory, attention concentration, logic thinking) were administered. Brain MRI was done in 44 (41.5%) BD patients. The study was conducted in the frames of the interdisciplinary program 'Stress factors and mental disorders in immune-mediated inflammatory rheumatic diseases'. RESULTS: CD of mild to moderate severity were diagnosed in 82 (77.4%) and anxiety-depressive disorders in 81 (76.4%) of BD patients. The patients with CD were older compared to patients without cognitive disorders (34.3±1.07 vs 29.0±2.14, p=0.006). Patients with CD were most often (84.1% vs 50.0%, p=0.001) diagnosed with anxiety-depressive disorder (anxiety, chronic/recurrent depression). MADRS scores were higher (16.1±0.74 vs 12.2±1.06, p=0.005) though did not exceed the moderate level. The impact of chronic psychosocial stressors was detected more often in CD patients. MRI results showed that the frequency of chronic multifocal, predominantly subcortical, changes in the white matter was higher in CD patients. CONCLUSION: CD are characteristic of most patients with BD. They are associated with the age, anxiety-depressive disorders, chronic stressors and minor brain multifocal subcortical parenchymal MRI lesions.

Ovarian function and disease activity in patients with systemic lupus erythematosus.

OBJECTIVE: Menstrual cycle disturbances frequently occur during the onset or in exacerbation periods of systemic lupus erythematosus (SLE), suggesting a possible relationship. The aim of the study is to assess the ovarian function in SLE patients with active disease before the treatment with high doses of glucocorticoids (GC) and cytotoxic agents. METHODS: We evaluated 94 female SLE patients (mean age of 29.2+/-7.0 years). The mean SLEDAI score was 11.4+/-8.1. Seventy-nine patients had a current use of GC with a median dose of 10 mg/day (8-15). The other 15 patients were untreated. After examination and blood sample collection 40% of the patients were treated and high doses of GC (>30 mg/day); 68% from this group of patients were treated GC in combination with cyclophosphamide (CYC). Forty healthy women with the same mean age were evaluated as controls. A careful gynecological history and a gynecological examination were carried out in patients and controls. Hormonal serum levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin, estradiol (E2) and progesterone in SLE patients and controls were measured by enzyme-linked immunosorbent assay (ELISA). RESULTS: Menstrual cycle disorders with oligomenorrhea as dominant aspect were observed in 54% of SLE patients. The hormonal studies showed decreased progesterone level in 52% of patients, reduced E2 concentration in 25% of patients; increased levels of LH, FSH and prolactin were observed with the lower frequency (13%, 9%, 10% respectively). Menstrual cycle disorders and the hormonal unbalance such as decreased progesterone level and hyperprolactinemia were found related significantly to high SLEDAI score (p<0.05, p=0.001, p<0.05). In the group of non-treated SLE patients the menstrual and hormonal disorders were observed in the same spectrum and with the same frequency as in all the examined SLE patients. SLEDAI score was found correlated significantly with the frequency of menstrual cycle disorders in non-treated SLE patients (p<0.05). CONCLUSION: The reported study shows the disease activity as a major factor associated with menstrual cycle disorders in SLE patients before treatment with alkylating agents and high doses of GC. Therefore, SLE women might be considered as a risk group for altered ovarian function.

[Behcet's disease].

The results of observation of 83 patients with Behcet's disease (BD) are represented. BD is included into the group of systemic vasculites with unknown etiology. Clinical manifestations of the disease are multiform. According to international criteria, suggested in 1990 year among examined by us BD patients the disease often is characterized by major diagnostic criteria: relapsing aphthous stomatitis, ulcerous lesion of genitals and ocular lesions. There are also small diagnostic criteria which include: disturbances of central nervous system (CNS), gastrointestinal tract (GIT), vascular pathology (arterial and venous thromboses), articular pathology e. a. The frequency of minor BD criteria is rather lower, but CNS and GIT disturbances and thromboses often determine the vital prognosis of the disease. We revealed that males suffer more often than females m:f = 1.7:1. Association between BD and HLA B5 antigen was found in 85.5% patients.

[Interleukin-18 in systemic lupus erythematosus: link with clinical symptoms and vascular atherosclerosis].

AIM: To examine correlation between IL-18 concentration, SLE manifestations and atherosclerotic vascular affection. MATERIAL AND METHODS: We examined 162 SLE female patients (age 26-43 years, mean age 35 years, duration of SLE 28-204 months, mean duration 96.0 months) and 64 healthy controls (women aged 30.0-45.0 years, mean age 35.5 years). We analysed correlation between IL-18 concentration, SLE symptoms, classic risk factors for cardiovascular diseases, subclinical and clinical signs of atherosclerosis. RESULTS: IL-18 in blood serum of SLE patients was much higher than in the control group (p < 0.00001). A positive correlation exists between IL-concentration and the disease activity by scale SLEDAI-2K, the level of antibodies to IgG cardiolipin, ESR, SLE, titers of antibodies to double-chain DNA, concentration of creatinin, urine, glucocorticoids dose taken by the patients at examination. In SLE patients with stomatitis, hematological, immunological disorders, a positive test for antinuclear factor, IL-18 was much higher than in patients without the above signs. No significant correlation was seen between IL-18 concentration, subclinical and clinical signs of atherosclerosis, Of risk factors, there was a negative correlation between total cholesterol concentration, LDLP and HDLP cholesterol. CONCLUSION: In SLE patients a high IL-18 level reflects activity of the basic disease and is not related with vascular atherosclerosis.

[Behcet's disease and joint affection].

AIM: To study joint pathology in patients with Behcet's disease (BD). MATERIAL AND METHODS: The trial enrolled 183 BD patients (128 males, 54females, mean age 32.0 +/- 9.8 years, age at BD onset 22.3 +/- 9.9 years, mean duration of the disease 9.5 +/- 8.9 years. RESULTS: Joint pathology occurred in 74.8% patients, in 10.9% patients the disease onset was associated with arthritis and arthralgia. 3 patients had aceptic necrosis of the head of the femur and knee joint, 3 patients--bone marrow infarction. Knee joints were affected more frequently than others (up to 40%) followed by ankle, elbow and radiocarpal and other joints. In BD duration about 10 years, none of the examinees had stage III of sacroiliitis, stage II was diagnosed only in 4 patients, stage I (suspicion)--in 9. Significant sacroillitis was not registered, bilateral sacroiliitis occurred only in 9 patients. HLA B5 was detected in 61% patients while HLA B27--only in 9.7%. CONCLUSION: The articular syndrome was seen in 3/4 of BD patients. Arthritis runs a recurrent course. Erosive arthritis, sacroiliitis and combination of the latter with HLA B27 are rare.

[Soluble CD40 ligand in systemic lupus erythematosus: link with atherosclerotic vascular affection].

AIM: To evaluate clinical implications of pCD40L as a marker of atherosclerotic vascular affection in systemic lupus erythematosus (SLE). MATERIAL AND METHODS: The examination of 132 females (mean age 35 years, SLE duration 96 months) assessed classic factors of atherosclerosis risk (AR), total coronary risk (TCR), detected subclinical atherosclerosis with ultrasonic scanning of the carotid arteries. Serum level of pCD40L was measured with enzyme immunoassay (EL4). RESULTS: Concentration of pCD40L in SLE patients was higher than in the control group (7.1 +/- 4.9 and 5.8 +/- 3.1 ng/ml, respectively) but the difference was insignificant (p > 0.05). Upper limit of normal value (M + 2SD) for pCD40L was 12 ng/ml. Elevation of pCD40L level was seen in 21% of SLE patients. Clinical manifestations of atherosclerosis risk were seen in 13% SLE patients, subclinical in 19 and 17% (increased intima-media thickness and atherosclerotic plaques, respectively). No correlations were found between pCD40L level and atherosclerotic symptoms. The pCD40L level was higher in SLE patients with the plaques than in those without them (p = 0.005). A positive correlation exists between a pCD40L concentration and maximal intima-media thickness (r = 0.2; p = 0.02), total cholersterol (r = 0.3; p = 0.002), LDLP cholesterol (r = 0.3; p = 0.004), LDLP cholesterol (r = 0.2; p = 0.04) and age of the patients (r = 0.2; p = 0.03). In patients with TCR > 20% a pCD40L was significantly higher than in patients with TCR < 20% (p = 0.01). CONCLUSION: Elevated pCD40L level is a marker of atherosclerotic affection of the vessels, has an important clinical role for predicting risk of cardiovascular diseases in SLE and elicidation of the role of activation of cell immunity in development of atherosclerosis in this disease.

[Clinical significance of heart rate variability in patients with systemic lupus erythematosus].

AIM: To evaluate clinical significance of heart rate variability (HRV) in patients with systemic lupus erythematosus (SLE). MATERIAL AND METHODS: HRV was investigated by means of time-domain analysis of 24 hour ambulatory ECG of 122 SLE patients under 55 years of age and 32 age-matched healthy controls. In addition to clinical manifestations and activity of SLE, we assessed the presence of basic conventional cardiovascular risk factors (hypertension, smoking, body mass index, dyslipidemia), performed common carotid duplex scanning with measurement of intima-medial thickness (IMT). Inflammatory markers (ESR, CRP, IL-6) were assessed in all the patients. RESULTS: Significantly lower HRV and the trend to tachycardia were detected in SLE patients when compared to the control group. There was a significant positive correlation between HRV and a cumulative dose of cyclophosphamide, a high density lipoprotein cholesterol level, a negative correlation between HRV and cumulative dose of azathioprine, standard risk factors (hypertension, smoking, body mass index, triglyceride level), markers of inflammation (ESR, CRP, IL-6) and IMT. CONCLUSION: Measurement of HRV in combination with routine cardiovascular risk factors and level of inflammatory markers can be used for identification of subjects at risk for faster progression of atherosclerosis in SLE patients.

[Catalytic autoantibodies as a new molecular instrument in rheumatological practice].

AIM: To compare clinicopathogenetic value of DNA-hydrolizing autoantibodies or DNA-abzymes in patients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). MATERIAL AND METHODS: We studied sera from 180 patients with SLE, 180 RA patients and 128 healthy donors matched by age and gender; assessed catalytic and cytotoxic activity of DNA-abzymes in patients with different variants of SLE and RA course. RESULTS: The highest catalytic and cytotoxic activities of DNA abzymes were observed in SLE patients. In SLE catalytic and cytotoxic activities of DNA abzymes ranged widely and their mean values depended on SLE activity in patients with systemic lesions. DNA-abzymes in RA patients showed lower catalytic and cytotoxic activities in relation to substrate DNA and target cells than in SLE. DNA-abzymes occurred most frequently in patients with high activity of RA, slow-progressive and lingering course of RA, especially in early development of visceral (extra-articular) pathology. Characteristic for DNA-abzymes in RA and SLE is the phenomenon of wide-range fluctuations due to factors determinating probability of induction of function of Ab-mediated catalysis and, therefore, incidence rates of DNA-abzymes, probably catalytic autoAb of the other specificity in a population of patients with systemic autoimmune diseases. CONCLUSION: The data indicate the validity of DNA abzymes use in clinical practice for realization of diagnostic and therapeutic programs in SLE and RA.

[Catalytic autoantibodies--a new molecular instrument in cardiology and ophthalmology].

AIM: To develop a conceptual model of using catalytic autoantibodies as diagnostic and monitoring tools in organ-specific autoimmune disorders. MATERIAL AND METHODS: A total of 99 patients (56 males and 43 females aged 21-52 years) with autoimmune myocarditis (AM) and 198 patients (77 males and 121 females aged 8-79 years) with autoimmune uveitis (A U) participated in the study. AM patients were examined for anticardiomyosin and anti-DNA autoantibodies (ACM, ADNAab), AU patients - for autoantibodies to S-antigen, IRBP, redopsin, phosphocine, autoDNA. RESULTS: AM patients had double level of DNA-binding autoantibodies. In 1/3 of them there was hydrolysing DNA and cytotoxic activity. In AU patients maximal titers were in Behcet's disease, sympathic ophthalmia, generalized uveitis and viral uveitis. CONCLUSION: Autoantibodies with different specificity and function including DNA-abzymes can be additional diagnostic and prognostic markers.

[The significance of cardiovascular risk factors and C-reactive protein to the development of atherosclerosis in women with systemic lupus erythematosus].

Compared with general population, women suffering from systemic lupus erythematosus (SLE) have signs of coronary artery disease (CAD) five to eight times more often, especially in young age. Early development of atherosclerosis in patients with SLE is caused by conventional cardiovascular risk factors and specific ones, associated with the disease and its therapy. A slight increase in such an inflammatory marker as C-reactive protein (CRP) is thought to reflect the presence of subclinical inflammation in the vascular wall, connected with atherosclerotic process. The authors analyzed the frequency of clinical and subclinical (an increase in the thickness of intima-media complex (IMC)) atherosclerotic manifestations, the summary coronary risk, the prevalence of conventional risk factors, and CRP level in 133 female patients with SLE and in 50 healthy donors. Compared to the control group, SLE patients were younger, developed cardiovascular diseases (CAD, stenocardia, myocardial infarction, and cerebral stroke (p = 0.05) as well as arterial hypertension more often, had higher levels of hs-CRP and triglycerides, and lower levels of high density lipoprotein cholesterol (HDLC). There was a positive correlation between hs-CRP level and the activity of the disease according to ECLAM score, ES value, IgG and IgM levels, hematological disturbances (anemia, leucopenia, and/or thrombocytopenia) , and a negative correlation with total cholesterol level, HDLC there was a moderate correlation between hs-CRP and a maximal IMC value.

[Neurological and psychiatric disorders in BehÒ«et's disease]. / Nevrologicheskie i psikhicheskie rasstroistva pri bolezni Bekhcheta.

The data of the literature on epidemiology, clinical manifestations, diagnosis and treatment of Behcet's disease are presented. Particular attention is paid to international recommendations (2014) on the classification, diagnostic criteria and treatment of neurological symptoms of BehÒ«et disease.

[Achievements and prospects of clinical abzymology].

Catalytic autoantibodies (abzymes) are autoantibodies that are potentially ready to realize certain effects in the organism, first of all antibody-mediated catalysis and cytotoxicity. Natural abzymes with protolytic (protabzymes) and DNA-hydrolyzing DNA-abzymes) activity are of the greatest interest. The most impressive example of the catalytic activity of protabzymes is hydrolysis of specific proteins, revealed in patients with autoimmune diseases, such as bronchial asthma (vasoactive intestinal neuropeptide), autoimmune thyroiditis (thyroglobulin), multiple sclerosis (myelin basic protein), and autoimmune myocarditis (cardiomyosin). The pathogenic role of DNA-abzymes is not quite clear yet. However, it has been proven that they present a powerful regulator of apoptosis and other cytotoxicity mechanisms in systemic autoimmune diseases and tumors. The most promising is use of abzymes as illness activity markers, and as therapeutic agents capable of catalyzing specific proteins or activating antitumoral chemotherapeutic preparations.

[Vascular symptoms of Behcet's disease].

AIM: To characterize vascular symptoms of Behcet's disease (BD) in patients treated for the last 10 years in the Institute of Rheumatology. MATERIAL AND METHODS: Vascular symptoms of BD were studied in 151 patients with BD (mean age 33.5 +/- 9.5 years, duration of the disease 14.0 +/- 10.1 years, 104 males and 47 females, 67.4% carried HLA B51(5) antigen). Duplex vascular scanning was made to detect venous thrombosis. RESULTS: Thrombotic complications were found in 37 of 151 (24.5%) patients with BD (30 males and 7 females, mean age 37.1 +/- 9.1 years, mean duration of the disease 7.3 +/- 9.7 years). Venous thromboses prevailed (36 patients). Four males (2.6%) had arterial lesions: aneurysms of the iliac arteries (n = 1), thrombosis of the iliac artery (n = 1), pulmonary infarction and thrombosis of the pulmonary arteries (n = 2). Combination of venous and arterial manifestations occurred in 3 patients. One patient had thrombosis of the venous sinus, occlusion of the retinal vessels was diagnosed in 7 patients. CONCLUSION: Incidence of vascular disorders was observed in 1/4 of the patients. This corresponds to those observed worldwide and concerns venous thrombosis and other thromboses. Among the examinees, vascular disorders were associated with a young age, earlier development of thrombotic complications in males than in females.