Objective: to assess the level of serum calprotectin (CLP) in Behcet's disease (BD).Material and methods. The study included 90 patients with BD (35 women and 55 men) and 30 healthy controls (22 women and 8 men). The mean age of the BD patients was 32 [26; 37] years, that of the control subjects was 30 [25; 37] years. Serum CLP levels were measured with an enzyme immunoassay using a reagent kit from Bulhmann Laboratories AG (Switzerland). Results and discussion. CLP levels were statistically significantly higher in patients with BD compared to healthy controls (median 4.08 [2.81; 7.25] vs. 2.86 [2.15; 3.92] μg/ml; p=0.003). Elevated serum CLP levels were found in 23 (26%) of the 90 patients with BD. Patients with high CLP levels were more likely to have active uveitis (odds ratio, OR 4.741; p=0.011), pustulosis (OR 3.41; p=0.044), arthritis (OR 13.89; p=0.014) and high BD activity (OR 3.195; p=0.029). A direct correlation was found between CLP level and BDCAF activity index (rs=0.415, p<0.0001), CRP (rs=0.466, p <0.0001) and ESR (rs=0.357, p=0.001).Conclusion. Serum CLP levels are elevated in patients with BD and are associated with high disease activity, active uveitis, pustulosis and arthritis.

Objective . To evaluate the ovarian reserve in women with systemic sclerosis (SSc) and to analyze the relationship of the concentration of anti-Müllerian hormone (AMH) with the main manifestations of the disease and therapy. Material and methods . The study included 74 SSc patients aged 18 to 40 years; the control group consisted of 32 healthy women, matched by age. The concentration of follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin, estradiol (E2) and testosterone was determined by enzyme immunoassay (ELISA), AMH quantitatively using standard chemiluminescent analysis on paramagnetic particles in blood serum. The AMG level of 1.0– 10.6 ng/ml was taken as normative values. Values <1.0 were regarded as a decrease in ovarian reserve. Results . In patients with SSD, the levels of AMH and testosterone were significantly lower than 1.4 [0.5; 2.3] and 0.45 [0.2; 0.96], respectively, versus 2.4 [1.8; 3.3] (p=0.002) and 1.6 [0.97; 2.5] (p=0.0001) in the control. The concentration of prolactin and E2 was recorded higher with SSDs – 22.23 [14.08; 31.18] and 140.2 [102.43; 179.74], respectively, against 10.2 [7.11; 16.68] (p=0.000002) and 95.3 [64.50; 130.0] (p=0.002) in the control. A decrease in the ovarian reserve by the level of AMH was significantly more often detected in patients with SSD in 43% versus 9.4% in the control (p=0.002). The risk of AMH reduction in patients with SSD was 7 times higher compared to the control (OR=7.030; 95% CI: 1.97–25.11). The levels of the hormones studied were comparable in patients with low and normal ovarian reserve. Diffuse form (46.9%) and subacute course of the disease (53.1%) were more often detected in patients with SSD and with low ovarian reserve compared to those with normal ovarian reserve (23.8% (p=0.033); 23.4% (p=0.004)). The frequency of organ lesions of SSDs, immunological disorders, inflammatory markers, and the lipid spectrum in the groups did not differ depending on the level of AMH. There were also no differences in the regimens and doses of treatment with basic anti-inflammatory drugs and glucocorticoids. Menstrual cycle disorders were noted by 31% of patients with SSD versus 6.2% in the control (p=0.004). Premature ovarian insufficiency (POI) was detected in 6.8% of patients with SSD and none in the control group (p=0.02). Patients with SSD and POI did not differ in age, duration of illness, clinical manifestations and therapy of them without POI. Conclusion . The concentration of AMH and testosterone was significantly lower in patients with. A decrease in ovarian reserve was significantly more often detected in women with SSs. Low ovarian reserve was more often detected in patients with diffuse form and subacute course of the disease. POI was more often observed in the group of SSc.

The aim – to evaluate the effectiveness of various immunosuppressive therapy schemes for current uveitis used in real clinical practice in patients with Behçet’s disease (BD).Material and methods. The study included 531 patients with a reliable (ICBD criteria 2014) diagnosis of BD, observed in the V.A. Nasonova Research Institute of Rheumatology from 2006 to 2020. The majority were men (331 (62.3%)). The average age (M±SD) was 32.9±10.0 years, the median duration of BD (Me (25%; 75%)) – 96 (48; 174) months. 60.4% patients had uveitis, 70.7% – exacerbation of uveitis (EU). Uveitis activity was assessed by the BOS24 index (Behçet’s disease Ocular attack Score 24) in 202 patients with EU. The total activity of BD was evaluated according to BDCAF index (Behçet’s Disease Current Activity Form). Glucocorticoids (GC) was systematically received by 68.7% patients with EU, including 51.5% in the form of pulse therapy. 88.9% patients with EU received cytotoxics: 33.5% – cyclosporine (CS), 20.7% – azathioprine (AZA), 11.4% – AZA+COL, 8.8% – AZA+CS, 7.5% – colchicine (COL), 3.9% – cyclophosphamide (CPh). 11.9% patients with EU were prescribe Biologics, mainly i-TNF-α (11,4%: 8,8% – adalimumab, 2.2% – infliximab, 0.4% – golimumab) and rituximab (0.4%). The effectiveness of therapy was evaluated on average after 18.0 (8.0; 36.0) months. Results . According to the dynamics of BDCAF, by the end of follow-up, BD activity significantly decreased in all groups, with the exception of patients who received COL. A more significant decrease in BDCAF was observed in the combination therapy groups: AZA+CS (ΔBDCAF=–4.08±3.60), AZA+COL (ΔBDCAF=–3.57±2.50), as well as in the CS group (ΔBDCAF=–3.57±3.39), but no statistically significant differences in ΔBDCAF between the groups were obtained, which does not allow us to speak about a significant advantage of a particular drug. There were no significant differences in ΔBDCAF between patients who received (ΔBDCAF=–3.41±3.89) and those who did not receive (ΔBDCAF=–3.59±3.23) Biologics. According to the dynamics of BOS24, the most effective for relieving symptoms of intraocular inflammation were CS (ΔBOS24=–7.0 (–12.0; –3.0)), AZA (ΔBOS24=–7.0 (–15.0; –2.0)), a combination of CS+AZA (ΔBOS24=–5.0 (–8.0; –2.0)) and CPh (ΔBOS24=–4.0 (–14.0; –2.0). The differences between BOS24 before and after treatment in these groups were statistically significant. When assigning AZA+COL (ΔBOS24=–1.0 (–4.0; 0)) or COL (ΔBOS24=–0.5 (–2.0; 0)) uveitis activity decreased during therapy, but not significantly. According to ΔBOS24, uveitis therapy by CS was statistically significantly more effective compared to AZA+COL and COL; and AZA treatment, compared to COL. Biologics, mainly adalimumab, significantly and rapidly reduce the severity of intraocular inflammation (ΔBOS24=–7.0 (–18.0; 0)) compared with GC and cytotoxics (ΔBOS24=–4,0 (–9,0; –1,0)), however statistically significant differences between the groups were not obtained due to the small number of Biologics groups. Conclusion . CS, AZA and their combination, as well as i-TNF-α (mainly adalimumab) are more effective for relieving uveitis symptoms in patients with BD. BOS24 is a reliable tool for quantifying the activity of uveitis in BD patients and its dynamics against the background of anti-inflammatory and immunosuppressive therapy.

Objective: to study the clinical and laboratory features of erythema nodosum (EN) in a cohort of patients with COVID-19 referred to a rheumatological center.Patients and methods. During 2020–2021 years 21 patients (18 women and 3 men, mean age 43.2±11.4 years) with EN and polyarthralgia/arthritis were examined. Depending on the time of EN and articular syndrome associated with COVID-19 development, patients were divided into three groups: 1) up to 4 weeks – acute COVID (symptoms potentially associated with infection); 2) from 4 to 12 weeks – ongoing symptomatic COVID and 3) more than 12 weeks – post-COVID syndrome (persistent symptoms not associated with an alternative diagnosis). All patients underwent a comprehensive clinical, laboratory and instrumental examination, including ultrasound of the joints and chest computed tomography (CT), as well as pathomorphological examination of skin and subcutaneous adipose tissue from the site of the node (in 9 cases). Results and discussion. Based on the anamnesis data, COVID-19 in the study cohort had mild (in 13 patients) and moderate (in 8) severity. Two patients (21 years old and 23 years old) with mild severity of the disease noted red painful (45 mm on the visual analogue scale of pain) nodes on the legs and polyarthralgia for the first time on the 2nd – 3rd day from respiratory symptoms onset. In 9 (52.3%) patients, mainly with a mild course, similar skin changes were detected 24.5±7.6 days after active COVID-19 relieve, i.e. during the period of ongoing symptomatic COVID. In 8 (38%) patients, including 6 with moderate severity of the disease, the appearance of nodes was noted after 85.6±12.3 days, which corresponded to the post-COVID syndrome.At the time of examination, complaints of skin rashes and joint pain were reported in 100 and 71.4% of patients, respectively. 67% of patients had shortness of breath, weakness, cough, sweating and myalgia. Subfebrile fever had 5 (24%) patients, mainly with ongoing symptomatic COVID (3 patients). In the overwhelming majority of cases (86%), EN was located on the anterior and lateral surfaces of shins, less often on the posterior and medial surfaces. It is noteworthy that the affection of more than 50% of the surface of the lower and upper extremities was associated with the number of nodes (p<0.02), the level of CRP (p<0.03) and the presence of post-COVID syndrome (p<0.2). Fifteen (71.4%) patients had arthralgias, mainly of ankle (80%) and knee (53.3%) joints.Laboratory abnormalities included: median ESR was 34 [12; 49] mm/h, CRP level – 9 [2; 32] mg/l. The results of the polymerase chain reaction for SARS-CoV-2 were negative in all patients. In 100% of cases IgG antibodies to SARS-CoV-2 were detected and in 52.3% – IgM antibodies. On chest CT 5% lung affection was detected in 43% of patients, 5–25% lesion in 57.1% of patients, 8 (38%) of whom were with post-COVID syndrome. Pathomorphological examination of the nodes showed signs of septal panniculitis.Conclusion. When EN, associated with SARS-CoV-2 appears it is important to suspect a post-infectious manifestation in time, based on the clinical picture of the disease and to determine the scope of further examination and adequate treatment.

Objective : to present cases of Behcet's disease (BD) concurrent  with axial spondyloarthritis  (axSpA). Subjects and methods . A total of 470 patients with BD who met the International Standard Bibliographic Description (ISBD) criteria were examined at the V.A. Nasonova Research Institute of Rheumatology in the period from 1990 to 2018. In 9 of them, BD was concurrent  with axSpA; 7 out of the 9 patients met the 1984 modified New York criteria for ankylosing spondylitis (AS) and two patients fulfilled the 2009 ASAS non-radiographic axSpA criteria. Results and discussion . Most (55.6%) patients were men. BD was preceded by axSpA in 6 patients. The symptoms of AS joined an average of 8.4 [2; 10] years after the onset of the clinical manifestations of BD. Three patients developed BB 9–15 years after the onset of the first symptoms of axSpA. The mean duration  of BD was 14.0±7.8 years and that of axSpA was 10.9±6.2  years. All the patients were HLA-B27-positive; three were found to have B5 antigen. All the 9 patients had mucocutaneous manifestations  that were characteristic  of BD. Four patients were diagnosed as having eye injuries: generalized uveitis with retinal vasculitis, posterior uveitis with or without retinal vasculitis. Gastrointestinal tract lesions were detected in 44.4% of cases. Peripheral  arthritis was present in all the patients. Entheseal involvement was observed in 66.7% of patients. The radiographic signs of bilateral sacroiliitis according to the modified New York criteria were found in 7 (77.8%) patients; MRI revealed active inflammatory changes in the area of the sacroiliac joint in 3 (33.3%) patients. Conclusion . The fact that there may be an overlap between BD and axSpA should be taken into account in everyday clinical practice, which will assist in choosing the optimal treatment strategy.

Objective: to compare the frequency of clinical manifestations of Behcet's disease (BD) in patients of both sexes in a Russian cohort.Patients and methods. Examinations were made in 425 patients (285 men and 140 women; mean age, 33.2±10.2 years; median duration of BD, 134.3 [60.0; 192.0] months (about 11 years) with a reliable diagnosis of the disease. 208 (48.9%) patients were ethnic residents of the North Caucasus. The activity of BD was assessed using the BD Current Activity Form; the disease severity was evaluated according to the classification proposed by Ch. Zouboulis.Results and discussion. In men with BD in the Russian cohort, the activity and severity of the disease were significantly higher and the HLA-B5(51)- antigen was more common. There was an association of male sex with thromboses in the deep cerebral veins and cerebral sinuses, damage to the eyes and skin (pseudopustulosis and pseudofolliculitis), genital ulcers, and a positive pathergy test. The findings are consistent with the results of studies of other patient cohorts: Iranian, German, Turkish ones, in which the men were more frequently detected to have damage to the vessels, skin and eyes.Conclusion. In the Russian patient cohort, the male sex is associated with high BD activity, severe organ damages, and HLA-B5(51)-antigen positivity, which is the basis for prescribing immunosuppressive therapy in men with early-stage BD.

Behсet's disease (BD) is a systemic vasculitis of an unknown etiology characterized by repeated oral and genital ulcerations and involvement of the eyes, joints, blood vessels, and other organs. In BD, it is the organ pathology that determines the severity and prognosis of the disease. The most difficult and least studied version of such changes is intestinal damage. Its verification in BD is complicated by a variety of clinical manifestations, their similarity with inflammatory bowel diseases, the lack of sufficiently informative laboratory tests, and pathognomonic endoscopic and histological signs. Intestinal BD (IBD) can result in serious complications (severe bleeding, intestinal perforation, and fistula formation), which may not only considerably reduce the quality of life of the patient, but also cause his death. Treatment for IBD is not standardized; it is mainly empirical and is performed as cycles. The goal of its therapy is to achieve clinical remission, to heal intestinal ulcers, to reduce recurrence rates, and to prevent surgery. The cumulative frequency of surgical interventions is as follows: 20% of them are performed in the first year, 27–33% within 5 years, and 31–46% within 10 years after IBD diagnosis. One of the problems in the surgical treatment of patients with IBD is the risk of a recurrence that often requires repeat surgery. Thus, 2- and 5-year postoperative recurrence rates were 29.2 and 47.2%, respectively. The poor prognostic factors are young age (<40 years), high disease activity at the time of diagnosis, detection of crateriform or deep ulcers at colonoscopy, high C-reactive protein level, and history of laparotomy.

Objective: to present cases of Behcet's disease (BD) concurrent with axial spondyloarthritis (axSpA).Subjects and methods. A total of 470 patients with BD who met the International Standard Bibliographic Description (ISBD) criteria were examined at the V.A. Nasonova Research Institute of Rheumatology in the period from 1990 to2018. In 9 of them, BD was concurrent with axSpA; 7 out of the 9 patients met the 1984 modified New York criteria for ankylosing spondylitis (AS) and two patients fulfilled the 2009 ASAS non-radiographic axSpA criteria.Results and discussion. Most (55.6%) patients were men. BD was preceded by axSpA in 6 patients. The symptoms of AS joined an average of 8.4 [2; 10] years after the onset of the clinical manifestations of BD. Three patients developed BB 9–15 years after the onset of the first symptoms of axSpA. The mean duration of BD was 14.0±7.8 years and that of axSpA was 10.9±6.2 years. All the patients were HLA-B27-positive; three were found to have B5 antigen.All the 9 patients had mucocutaneous manifestations that were characteristic of BD. Four patients were diagnosedas having eye injuries: generalized uveitis with retinal vasculitis, posterior uveitis with or without retinal vasculitis. Gastrointestinal tract lesions were detected in 44.4% of cases. Peripheral arthritis was present in all the patients. Entheseal involvement was observed in 66.7% of patients. The radiographic signs of bilateral sacroiliitis according to the modified New York criteria were found in 7 (77.8%) patients; MRI revealed active inflammatory changes in the area of the sacroiliac joint in 3 (33.3%) patients.Conclusion. The fact that there may be an overlap between BD and axSpA should be taken into account in everyday clinical practice, which will assist in choosing the optimal treatment strategy.

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.

The lecture considers in detail modern ideas about the epidemiology, pathogenesis, and clinical manifestations of Behcet's disease/syndrome, as well as the principles of diagnosis, assessment of the activity and severity of the disease, and approaches to therapy.

Введение. Болезнь Бехчета (ББ) – системный васкулит неизвестной этиологии, характеризующийся рецидивами язвенного процесса в ротовой полости и на гениталиях, частым поражением глаз и рядом проявлений, включающих патологию суставов, желудочно-кишечного тракта, нервной системы и сосудов. Цель исследования: определить частоту и локализацию сосудистых проявлений у больных с ББ, уточнить связь последних с полом, возрастом и другой клинической симптоматикой ББ. Материалы и методы. Обследовано 250 больных ББ, сосудистая патология выявлена у 63 (25,2%) пациентов. Возраст больных с сосудистыми проявлениями составил 34,8 ± 10,4 лет, соотношение мужчины:женщины – 3,5:1. Сосуды вовлекались в процесс чаще в течение первых трех лет болезни. Поражалось в основном венозное русло, доля больных с артериальной патологией составила 9,5%. Результаты. У пациентов с сосудистой патологией по сравнению с больными без тромбозов достоверно чаще выявлялись узловатая эритема (71% против 44%; р = 0,002) и эпидидимит (22,4 % против 10,9%; р = 0,04), а генерализованный увеит (17,4% против 32%; р = 0,002) и ангиит сетчатки (22% против 40,6%; р = 0,008) встречались достоверно реже. В системе коагуляции у больных ББ с и без сосудистой патологии достоверных различий не выявлено. Заключение. Сосудистые нарушения выявлены у ¼ больных (преимущественно мужчин молодого возраста), развивались чаще в первые три года болезни и ассоциировались с узловатой эритемой, эпидидимитом и положительным тестом патергии. Introduction. Behcet's disease (BB) is a systemic vasculitis of unknown etiology; it is characterized by recurrent aphthous stomatitis, genital ulcers, uveitis and other manifestations including arthritis, pathology of gastrointestinal tract, nervous system and blood vessels. The aim of study: to determine frequency and localization of vascular manifestations in patients with BB, to clarify the relationship of manifestations with sex, age and other clinical symptoms of BB. Materials and methods. We examined 250 patients with BB, vascular pathology was revealed in 63 (25,2%) patients. The age of patients with vascular manifestations was 34,8 ± 10,4 years, the ratio of men:women was 3,5:1. Vessels were involved in the process more often during the first three years of the disease. Mainly veins were affected, arterial pathology had 9,5% of patients. Results. In patients with vascular pathology erythema nodosum (71% vs. 44%, p = 0,002) and epididymitis (22,4% vs. 10.9%; p = 0,04) were significantly more frequent than in patients without thromboses, and panuveitis (17,4% vs. 32%; p = 0,002) and retinal vasculitis (22% vs. 40,6%; p = 0,008) were found significantly more rarely. There were no significant differences in coagulation system between patients with and without vascular pathology. Conclusion. Vascular disorders were detected in ¼ of patients (predominantly in men of young age), developed more often in the first three years of the disease and associated with erythema nodosum, epididymitis and a positive pathergy test.

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The paper considers the major effects of cyclosporin A (CsA) in rheumatic diseases (RD). CsA is noted to be effective for a number of RDs and all types of rheumatoid arthritis (RA), including in early RA in adults and juvenile RA; uveitis associated with Behcet's disease (BD) (as the drug of choice); in combination with other disease-modifying drugs; in RD with concomitant viral hepatitis C. The diagnostic criteria for (BD) and the efficacy of CsA for this disease are given according to different authors.

Objective: To study the incidence of vascular disorders and to estimate their contribution to the severity and prognosis of Behcet's disease (BD). Subjects and methods. Ninety-five patients with evident BD, followed up at Research Institute of Rheumatology, Russian Academy of Medical Sciences, in the January 2006 to October 2009, were examined. Their mean age was 29,7 years; malefemale ratio was 3,7. The onset of the disease was chiefly at the age of 21-30 years in 36,8% of the patients. Vascular pathology was evaluated by the following techniques: vascular duplex scanning; brain magnetic resonance imaging in the mode of venosinusography, contrast-enhancement chest computed tomography, chest and abdominal angiography. The data were statistically processed by the programs Statistica 6.0 (Statsoft, USA) and EpiInfo 5.0 recommended by the WHO. Parametric and non-parametric statistical methods were used. Results. Vascular diseases were diagnosed in 26,3% of the patients. The venous bed was mainly involved (88%), the proportion of patients with arterial pathology was 12%. Vascular death occurred in 2 men aged 20 and 18 years due to pulmonary artery rupture aneurysm and brain sagittal sinus thrombosis. Assessment of an association of vascular disorders with other clinical manifestations of BD revealed their correlation with erythema nodosum and epididymitis. The risk for vascular events in erythema nodosum was 5 times greater (OR=5,03; 95% CI 1,54-17,41), the risk was higher in men than that in women (OR=7,11; 95% CI 1,83-30,02 versus OR=2,15; 95% CI 0,16-61,38). The male vascular risk is associated with epididymitis (OR=6,71; 95% CI 1,25-39,89). In erythema nodosum concurrent with epididymitis, the relative vascular risk was increased up to 21; 95% CI 2,15-503,94. Conclusion. Vascular disorders in BD were diagnosed in one fourth of the patients, mainly in young male patients. Severe thromboses with the development of chronic venous insignificance, Budd-Chiari syndrome, pulmonary and iliac artery aneurysms, and arterial thromboses were observed in male patients only. Vascular events were associated with erythema nodosum and epididymitis; in these concomitances, the vascular risk was substantially increased. Vascular death rates were 2,2%.

Behçet’s disease (BD) is a systemic vasculitis of unknown origin characterized by ulcerous damage of oral and genital mucosa as well as eyes and skin. Other organs (nervous system, lungs, joints, gastro-intestinal tract) are rarely involved. Damage of vessels of all sizes and types is a feature of this vasculitis. Review of main lung BD manifestations (pulmonary artery aneurisms, arterial and venous thrombosis, parenchymatous lung damage) and own description of a pt with BD with multiple pulmonary aneurisms are presented.

Behcet’s disease (BD) is a systemic vasculitis of unknown origin characterized by ulcerous damage of oral and genital mucosa as well as eyes and skin. Other organs (nervous system, lungs, joints, gastro-intestinal tract) are rarely involved. Damage of vessels of all sizes and types is a feature of this vasculitis. Review of main lung BD manifestations (pulmonary artery aneurisms, arterial and venous thrombosis, parenchymatous lung damage) and own description of a pt with BD with multiple pulmonary aneurisms are presented.

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