Uncontrolled activation of neutrophils is considered an important mechanism of thromboinflammation and fibrosis in immunemediated rheumatic diseases (IMRD), malignant neoplasms, atherosclerosis, COVID-19 and many other acute and chronic inflammatory diseases of humans. Particular attention has been drawn to the ability of neutrophils to form “network” (web-like) structures, called “neutrophil extracellular traps” NETs. The process associated with the formation of NETs and the weakening of their degradation is called “NETosis”. The publication summarizes data on the role of NETosis in the pathogenesis of IMRD and discusses the prospects for pharmacotherapy aimed at preventing the formation and destruction of NETs.

The new 2022 classification criteria for antineutrophil cytoplasmic antibodies associated vasculitis (AAV), proposed by the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR), was an important stage in the development of the doctrine of systemic vasculitis. Based on the analysis of large international cohorts, the criteria for granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis were revised, and classification criteria for microscopic polyangiitis were proposed for the first time. The creation’s history of the AAV classification criteria is discussed over the past three decades, and their limitations are considered. It should be noted, the ACR/EULAR 2022 criteria were developed for creating homogeneous groups of patients for scientific studies and cannot be widely used in clinical practice for the various AAV nosological forms diagnosis, primarily at early stage of the disease or mono organ damage (for example, kidneys). As before, decisive importance in AVV diagnostic belong to a detailed clinical analysis and a complete examination of patients to identify pathognomonic symptoms, including asymptomatic ones.

The aim of the study was to assess frequency and severity of COVID-19 in patients with rheumatic diseases (RD) who were on inpatient treatment at the V.A. Nasonova Research Institute of Rheumatology.
Patients and methods. The study included information on the presence or absence of COVID-19 in the medical history of 6911 patients with immunoinflammatory RD (IIRD) and 362 patients with osteoarthritis (OA) who were on inpatient treatment at the V.A. Nasonova Research Institute of Rheumatology from September 21, 2021 to April 28, 2023.
Results. The incidence of COVID-19 in the analyzed IIRD was significantly higher compared to OA (p<0.001). All IIRD included in the analysis are characterized by an increased risk of COVID-19 incidence when compared with OA by 2.7–6.3 times. Patients with rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, microcrystalline arthritis, Sjögren’s disease, systemic sclerosis, systemic lupus erythematosus, and ANCA-associated vasculitis were significantly more likely (p<0.02) to receive COVID-19 therapy compared with the control group. Patients with these diseases are characterized by an increased risk of treatment for COVID-19 by 1.9–3.7 times compared with OA. Also, patients with inflammatory joint diseases (IJD), connective tissue diseases (CTDs) and systemic vasculitis (SV) were hospitalized with COVID-19 more often than patients with OA (p=0.01, p=0.007 and p=0.024, respectively). Patients with IJD, CTDs and SV are characterized by an increased risk of hospitalization with COVID-19 by 4.3– 4.7 times compared with OA. In addition, elderly patients with IIRD are characterized by an increasing risk of treatment, hospitalization and use of biologics or targeted synthetic disease-modifying drugs for COVID-19.
Conclusion. According to the results obtained, the problem of COVID-19 is significant for patients with RD, which dictates need for further research on vaccination against SARS-CoV-2 among this cohort of patients.

Rituximab is a chimeric (mouse and human) monoclonal antibody against B-lymphocytes (CD20). This drug is widely used in rheumatology in the treatment of rheumatoid arthritis, Sjogren’s syndrome, some systemic connective tissue diseases and vasculitis, as well as in hematology in lymphoproliferative diseases. Administration of rituximab leads to depletion of B-lymphocytes through various mechanisms, including antibody-dependent and complementdependent cytotoxic effects, as well as the regulation of apoptosis. Considering the mechanism of autoimmune damage in Graves’ disease, an autoimmune thyroid disease accompanied by thyrotoxicosis and endocrine ophthalmopathy (an autoimmune lesion of the accessory apparatus of the eye), the use of rituximab may be effective in these diseases. The review considers the currently available results of studies on the use of various doses of rituximab in these diseases.

The aim of the study was to evaluate in real clinical practice the effectiveness of therapy with an interleukin-6 inhibitor (olokizumab) in patients with rheumatoid arthritis (RA) in terms of clinical and laboratory activity of the disease, as well as patient-reported outcomes (PROs).
Material and methods. 10 patients with a reliable diagnosis of RA were examined: the average age of the patients was 45.70±17.9 years, the duration of the disease was 9.0 (3.0; 12.0) years. Patients were with moderate or high disease activity: DAS28-ESR – 5.13 (4.34; 5.80) points; CDAI – 30.00 (24.00; 35.00); SDAI – 31.86 (24.36; 38,59). All patients were prescribed treatment with olokizumab (OKZ) at a dose of 64 mg subcutaneously every 4 weeks against the background of therapy with methotrexate, leflunomide, non-steroidal anti-inflammatory drugs and glucocorticoids (GC) (up to 10 mg/day in terms of prednisolone). Three patients had previously received tocilizumab (intravenously once a month at a dose of 8 mg/kg), the administration of which was discontinued for administrative reasons 6–12 months before the appointment of OKZ.
The results of treatment were assessed by the dynamics of clinical, laboratory parameters (DAS28-ESR, SDAI, СDAI, CRP, ESR, IgM RF, ACCP) and outcomes assessed by the patients themselves (PROs): HAQ-DI index, general assessment of the health status of patients (OSZB) according to VAS, pain according to VAS; scales FACIT, SF-36. As psychometric methods, the questionnaire “Type of attitude towards the disease (TOBOL)”, the Hospital Anxiety and Depression Scale (HADS), and the Toronto Alexithymic Scale (TAS-26) were used. Observation was carried out before treatment, after 3 and 6 months of therapy.
Results. Against the background of OKZ therapy, after 3 and 6 months, compared with the baseline, there was a significant decrease in the clinical indices of RA activity: DAS28-ESR – 5.13 (4.34; 5.80), 3.53 (2.83; 4.26) and 3.48 (2.8; 4.10) points respectively; CDAI – 30.00 (24.00; 35.00), 11.00 (6.0; 16.00) and 10.0 (5.0; 15.0) points respectively; SDAI – 31.86 (24.36; 38.59), 11.05 (6.07; 16.07) and 10.17 (7.02; 15.02) points respectively; CRP – 14.30 (7.00; 24.70), 0.70 (0.40; 0.90) and 0.65 (0.20; 3.0) mg/l respectively. No significant dynamics of ESR, RF IgM and ACCP was noted.
After 3 and 6 months of treatment with OKZ, there was a significant decrease in OSZB and pain severity according to the VAS scale, and an improvement in the functional state of patients was observed according to the HAQ-DI questionnaire of fatigue indicators (FACIT-F) (p<0.05). The physical component of the SF-36 scale increased significantly only by the 6th month of therapy (p<0.01), while the mental component did not undergo significant changes (p>0.05). In the process of treatment of OKZ, the attitude of patients to the disease changed from the ergopathic, neurasthenic and sensitive components in the TOBOL profile, before it began, then by its end, the dominant ones were harmonious, ergopathic and sensitive profiles. In addition, starting from the 3rd month of treatment in patients with RA, an adaptive response to the disease prevailed. A decrease in the level of anxiety was revealed, compared with the baseline, after 3 and 6 months of observation, depression indicators did not change significantly.
Conclusion. In general, the results of this study indicate the effectiveness of ICD in RA, not only in terms of reducing the clinical and laboratory activity of the disease, but also in terms of outcomes reported by the patient himself, characterizing the quality of life and the psycho-emotional state of patients.

Background. Gout is associated with increased risk of cardiovascular disease (CVD) morbidity and mortality. Therefore, an association between coronary heart disease (CHD) and gout deserves careful examination.
The aim of this study was to determine the prevalence of CHD and factors associated with CHD in patients (pts) with gout.
Methods. 286 male patients with gout were included; age – 51.2 [42.8; 59.4] years (ys), disease duration – 6.2 [3.8; 12.1] ys. All patients underwent standard clinical examination, screening traditional risk factors (TRF) of CVD. We estimated the adjusted odds ratio (OR) and 95% confidence interval (95% CI).
Results. CHD was found in 111 out of the 286 pts (38.8%), MI had a history in 29.7%. Compared to individuals with CHD, participants without CHD were older (56.7 [52.1; 61.1] vs 46.2 [40.6; 53.4] ys), had longer duration of gout (9.3 [4.7; 15.1] vs 5.6 [3.3; 9.7] ys) (for all p<0.05). Abdominal obesity (OR=3.6; 95% CI: 1.2–10.9), family history of CHD (OR=2.2; 95% CI: 1.3–5.4), disease duration of gout more 10 ys (OR=2.8; 95% CI: 1.6–4.7), age of gout onset <35 ys (OR=5.5; 95% CI: 2.6–11.7), intraosseous tophi (OR=3.03; 95% CI: 1.8–5.01), nephrolithiasis (OR=1.7; 95% CI: 1.04–3.04), renal failure (OR=5.6; 95% CI: 2.7–11.4), serum total cholesterol (TC) (OR=1.6; 95% CI: 1.0–2.8), serum creatinine (OR=2.5; 95% CI: 1.2–5.1), increased the risk for CHD in patients with a gout.
Conclusions. The prevalence of CHD was 38.8% among individuals with gout (third of patients had a history of MI 29.7%). Our study showed that both TRFs of CVD and the severity of gout and a history of renal failure contribute to the development of CHD in patients with gout.

The aim – to establish the frequency of frailty and the ratio of its main phenotypic features in patients with rheumatoid arthritis (RA), as well as to identify associated factors.
Material and methods. The study included 101 patients (86 women and 15 men) with RA at the age of 60 [52; 66] years; the average duration of the disease – 8 [3; 15] years. Frailty syndrome was diagnosed by the phenotypic model of L.P. Fried et al. In addition, the strength of the quadriceps femoris muscle was assessed using the test of getting up from a chair, functional status according to HAQ-DI (Health Assessment Questionnaire – Disability Index); Charlson comorbidity index, presence of cardiovascular disease (CVD); dementia severity and nutritional status. Factors associated with frailty were identified by the principal component method with the rotation of the correlation matrix using the Varimax raw method.
Results. Frailty syndrome was detected in 40.6%, prefrailty in 55.4% of patients with RA, robust patients were 4.0%. The dominant signs of frailty in RA patients are reduced hand strength, fatigue, and weight loss. Four clusters of factors associated with frailty syndrome in RA have been identified: reduced hand strength, HAQ-DI impairment, and RA activity; reduced survival, comorbid cardiovascular disease and age; the total dose of glucocorticoids (GC) taken, an increase in walking time by 4 m and hypodynamia; eating disorders.
Conclusion. Frailty and prefrailty dominate in patients with RA and have certain phenotypic features. Factors associated with frailty and it’s diagnostic features include impairment of vital activity, RA activity; premature mortality, CVD comorbidity, older age; taking GCS and malnutrition.

Damage to the cardiovascular system (CVS) in Takayasu arteritis (AT) is characterized by a wide range of clinical manifestations and an unfavorable prognosis of the disease.
Objectives: determine the characteristics of clinical manifestations of damage to the CVS and predictors of unfavorable prognosis in Kyrgyz patients with АТ.
Methods: The study included 135 patients with a reliable diagnosis of AT, verified according to the classification criteria of the American College of Rheumatology/The European Alliance of Rheumatology Associations (ACR/EULAR, American College of Rheumatology/European Alliance of Associations for Rheumatology). All patients underwent clinical and standard laboratory and instrumental examination.
Results: CVC lesion was observed in 84.4% of patients with AT and was manifested mainly by secondary arterial hypertension (AH) and aortic regurgitation (AR) – 52.8% and 27.8% of cases, respectively. The main cause of secondary hypertension was renovascular hypertension (63.4%). Myocarditis (8.5%) and coronary syndrome (3.8%) were significantly less frequently diagnosed. Among the cardiovascular complications (CVc) that developed in 32.5% of patients, decompensated chronic heart failure (DCHF) (48.7%) and acute cerebrovascular accident (ACVA) were the most common (40,5%). The immediate cause of death in the observed cohort was DCHF (66.7%) due to severe AR (83.3%) and ischemic cardiomyopathy (16.7%). Predictors of the development of CVc were the V anatomical type of vascular lesion, high activity of the pathological process, severity of stenotic changes, serious clinical complications (CC) and severe AR (p<0.05).
Conclusion:
1. CVS lesion was observed in 84.4% of Kyrgyz patients with AT.
2. The most frequent variant of cardiovascular pathology was secondary hypertension (52.8%) caused by vasorenal (63.4%), coarctation (33%) and aortic (3.6%) changes. The second most common was pathology of the aortic valve (27.8%) with a predominance of minor AR (45.8%).
3. CVc was observed in one third of patients (32.5%) and in most cases were presented with DCHF (48.7%) and ACVA (40.5%).
4. Unfavorable factors associated with an increased risk of CVc in the observed patients were V anatomical type of vascular lesion, high activity, pronounced stenotic changes, severe CC and severe AR (p<0.05).

Background. Remission/low disease activity (LDA) are acceptable goal of psoriatic arthritis (PsA) treatment. Prognostic factors for non-remission/LDA hasn’t been fully studied yet and data is limited.
The aim – to determine the prognostic factors associated with non-remission/LDA status within 1 year of treatment in PsA pts in real practice. Methods. 292 pts (M/F=122/170) with active PsA fulfilling the CASPAR criteria were included. Mean age 46.1±12.5 years (yrs), PsA duration 10.4±7.1 months (mos), psoriasis (Ps) duration 19.32±12.08 mos, body mass index (BMI) 27.7±5.6 kg/m² , median (Me) of DAPSA – 23.8 [14.7; 37.4]. 182 pts was given therapy with synthetic (s) DMARDs predominantly methotrexate (MTX), 110 pts – bDMARDs as monotherapy or with combination with MTX or other sDMARDs. At baseline (BL) and at 1 year of therapy PsA activity by tender/swelling joint count (TJC)/68, (SJC)/66, pain (VAS), Patient global assessment disease activity (PtGA, VAS), CRP (mg/l), dactylitis, enthesitis by LEI and plantar fascia, BSA (%), HAQ, DAPSA were evaluated. DAPSA>28 indicate high disease activity (HDA), DAPSA=15–28 – moderate activity (MoDA), DAPSA=5–14 – LDA, DAPSA≤4 – remission. By 1 year of therapy the proportion of pts who had not reached remission or LDA were calculated. The one-factor model of logistic regression was used to identify a group of features that are associated with remission or LDA  nonachievement. M±SD, Me [Q25; Q75], Min–Max, %, t-test, Peаrson χ² , Mann – Whitney tests, ORs with 95% CI were performed. All p<0.05, were considered to indicate statistical significance.
Results. At 1 year of therapy 116 pts of 292 (40%) have HDA/MoDA by DAPSA. Remission/LDA was reached in 176 (60%) pts, 110 of them (62.5%) were treated with bDMARDs. Comparative analysis in both groups and one-factor model of logistic regression showed the following features at BL were associated with non-remission/LDA status: TJC>5 (p<0.001), SJC>3 (p<0.001), CRP>10 mg/l (p<0.001), HAQ>0.5 (p<0.001), presence of enthesitis (p<0.001), dactylitis (p<0.001), BMI>30 (p<0.002) and had to be treated with sDMARDs. PsA pts with combination of these clinical features at first visit have a higher risk of not achieving remission/LDA status in comparison to PsA pts without them, OR with 95% CI.
Conclusion. In real practice remission/LDA cannot achieve 40% PsA pts despite going through therapy. It is a combination of clinical fea tures at BL – TJC>3, SJC>5, CRP>10 mg/l, HAQ>0.5, presence of enthesitis, dactylitis, BMI>30 kg/m² and sDMARDs monotherapy – that constitutesa prognostic factor with negative impact on achievement remission/LDA after 1 year of treatment.

Background. Biological disease-modifying antirheumatic drugs (bDMARDs) can have different effects on various clinical manifestations of ankylosing spondylitis (AS). Data on the effects of interleukin 17 inhibitors (iIL17) on uveitis in AS continue to accumulate.
Objective – to evaluate the effect of iIL17 therapy on the course of uveitis in AS.
Material and methods. 73 patients (pts) with AS (New York criteria, 1984), who received iIL17 (57 – secukinumab (SEC), 22 – netakimab (NTK)) for at least 1 year were included in the study. The average age of pts at the time of inclusion in the study was 41.93±8.95 years, the average duration of AS was 10.75±6.22 years. There were 40 (56.7%) men and 33 (43.3%) women among the pts. HLA-B27 was detected in 62/73 (85%), coxitis in 58 (79%), enthesitis in 63 (86.3%), peripheral arthritis in 57 (78%), psoriasis in 7 (9.5%), inflammatory bowel disease (IBD) in 3 (4.1%); in 6 (8.2%) the disease started before the age of 16; 19 (26%) pts had at least 1 episode of uveitis during the course of the disease.
The rates of uveitis was estimated by comparing the number of incidences per 100 patient-years before the start of bDMARDs therapy and during iIL17 using.
Result. The incidence rate of uveitis before the start of bDMARDs therapy for all pts was 8.3 per 100 pt-years (95% CI: 0.065–0.107), during iIL17 therapy – 9.2 per 100 pt-years (95% CI: 0.06–0.15; p=0.72).
The incidence rate of uveitis among pts used SEC was 10.1 per 100 patient-years (95% CI: 0.079–0.13) before the start of bDMARDs therapy, during SEC using – 9.4 per 100 pt-years (95% CI: 0.05–0.15; p=0.74).
The incidence rate of uveitis among pts used NTK was 4.8 per 100 pt-years (95% CI: 0.028–0.08) before the start of bDMARDs therapy, during the NTK using – 7.1 per 100 pt-years (95% CI: 0.019–0,22; p=0.3).
For patients with a history of uveitis, the incidence rate of uveitis before the start of therapy with bDMARDs was 22.5 per 100 pt-years (95% CI: 0.18–0.28), during iIL17 therapy – 29.1 per 100 pt-years (95% CI: 0.18–0.43; p=0.29).
Occurrences of uveitis were observed in 4 of 57 pts (7%) during the using of SEC, and in 1 of 25 pts (4%) – during the NTK therapy. 1 case of new-onset uveitis was recorded during the using of SEC.
Conclusion. There were no significant differences in the incidence rates of uveitis during iIL17 using compared with non-biological therapy. iIL17 have not demonstrated a significant effect on the course of uveitis in AS in the study group. 

Introduction. Currently, there is a small number of studies devoted to the differences in the manifestations of axial spondyloarthritis (axSpA) in men and women, and there are no studies assessing the frequency of coxitis in axSpA in patients of different sexes.
The purpose of the study is to compare the main manifestations of coxitis in axial spondyloarthritis in men and women.
Material and methods. The study was conducted on the Moscow Cohort of Early SpondyloArthritis (CoRSAr), which currently includes 175 patients. Their age at the time of inclusion in the cohort was on average 28.5±5.8 years, the duration of the disease was 24.1±15.4 months. 92.6% of patients were positive for HLA-B27 (human leukocyte antigen B27). All patients were assessed for pain in the hip joints (HIP) using a numerical rating scale (NRS) (0–10), and pelvic radiography, magnetic resonance imaging (MRI) and ultrasound examination of the Hip joint were performed. An increase in the cervical-capsular distance (NCD) of more than 7.0 mm was considered a sign of coxitis according to ultrasound data. When analyzing MRI results, signs of coxitis were considered to be synovitis and/or osteitis of the femoral head or acetabulum. X-ray changes in the hip joint were assessed using the BASRI-hip index (Bath Ankylosing Spondylitis Radiology Hip Index).
Results and discussion. Among the 175 patients, there were 97 (55.4%) men and 79 (44.6%) women. At the time of inclusion in the study, women were older than men (p<0.01), while the duration of the disease in men and women was the same and averaged about 2 years. In men, X-ray changes in the hip joint were more common than in women (in 7.2% and 1.3% of cases, respectively; p<0.05). Clinical signs of coxitis were detected in 54 (55.7%) men and 41 (51.9%) women, while the level of pain according to the NRS in women averaged about 2, and in men – about 3 points (p>0.05). According to ultrasound data, coxitis was detected in 15 (18.9%) women and 27 (27.8%) men; NCD averaged 6.2 mm in men and 5.9 mm in women (p>0.05). In men, signs of damage to the hip joint according to MRI were more common than in women (in 27.8% and 15.1% of cases, respectively; p<0.05). The level of C-reactive protein (CRP) in men was also higher than in women (on average 12.8 and 4.3 mg/l, respectively; p<0.05).
Conclusion. Women develop axSpA at a later age than men, and the latter are more likely to have HLA-B27, MRI features of coxitis, and higher levels of CRP.

The aim – to assess bone mineral density (BMD) and microarchitecture, as well as the risk of fractures in postmenopausal women with rheumatoid arthritis (RA).
Materials and methods: 95 postmenopausal women (mean age 62.3±8.1 years) with a confirmed RA were included. All patients underwent a questionnaire, clinical and laboratory examination, dual-energy X-ray absorptiometry (DXA) of the lumbar spine (L1–L4), proximal femur, and trabecular bone score (TBS) assessment. The 10-year probability of osteoporotic fracture was calculated using the FRAX tool without including femoral neck (FN) BMD (BMD–), with FN BMD (BMD +) and additionally adjustment for TBS (BMD + TBS).
Results. Osteoporosis (OP) was found in 41 (43.2%) patients: in L1–L4 – in 26,3%, in FN – in 22.1%, and in the total hip (TH) – in 11.6% persons. Degraded microarchitecture according to TBS was found in 38.9% of patients, partially degraded – in 25.3%, and normal – in 35.8% of women with RA. A high risk of fracture according to FRAX BMD– was detected in 49.5% of patients. TBS correlated with age (r=–0.30; p=0.003), duration of postmenopausal period (r=–0.26; p=0.014), cumulative dose of glucocorticoids (GCs) (r=–0.34; p=0.045), FRAX BMD– (r=–0.24; p<0.05) and FRAX BMD+ (r=–0.21; p<0.05); L1–L4 BMD (r=0.43; p<0.001), FN BMD (r=0.21; p=0.038), TH BMD (r=0.23; p=0.02). Low TBS was significantly more often detected in people with a history of fractures compared to people without them (p<0.05). Among RA patients with normal L1–L4 BMD 9.5% of persons had degraded microarchitecture of bone tissue according to TBS. The inclusion of TBS in FRAX increased the risk of fractures to high in 9.5% of patients and reduced it to low in 7.4% of women, due to which the total number of people with RA who had a high risk of fractures became 54.7%.
Conclusion. OP was diagnosed in 43.2%, and degraded microarchitecture of bone tissue according to TBS – in 38.9% of postmenopausal women with RA. A high risk of fractures according to FRAX was found in 49.5%. TBS negatively correlated with age, duration of postmenopause, cumulative GCs dose, FRAX fracture risk, and positively correlated with BMD in all measurement sites. The FRAX adjustment by TBS redistributed patients in risk groups, as a result of which 54.7% of RA patients needed anti-osteoporotic treatment.

Introduction. Juvenile idiopathic arthritis (JIA) is a common multifactorial disease characterized by the presence of chronic inflammation in the joints, entheses and other structures of the musculoskeletal system in combination with a certain range of extraskeletal disorders. Vast variety of JIA clinical variants and the variability of the disease course make primary and differential diagnosis difficult, which often leads to a delayed start of treatment and an inadequate choice of medical therapy or, conversely, an excess of medication. In the range of differential diagnostic conditions that have similar symptoms and are manifested by severe arthralgia, gait disturbance, joint stiffness, as well as the presence of effusion and gradual progression of bone destruction mainly in the epiphyseal plate, one should remember about hereditary skeletal dysplasias, primarily from a genetically heterogeneous group of multiple epiphyseal dysplasias (MED).
The aim of the study – description of the clinical and genetic characteristics of three patients with various genetic variants of MED and defining approaches for their differential diagnosis with JIA.
Materials and methods. There were three patients from three unrelated families aged from 7 to 13 years old under our supervision. To clarify the diagnosis, a genealogical analysis, a clinical examination of patients and first-degree relatives, as well as an assessment of X-ray images of long tubular bones were carried out. Molecular genetic confirmation of the MED diagnosis types 1 and 2 was based on the results of custom panel sequencing consisting of 166 genes responsible for the development of hereditary skeletal pathology. To clarify the molecular genetic diagnosis of MED type 4, an analysis of the SLC26A2 gene was performed using automated Sanger sequencing.
Results. Anamnestic, clinical, radiological, and molecular genetic characteristics of three unrelated patients with different genetic types of MED caused by variants in the COMP, SLC26A2, and COL9A2 genes were analyzed. The first symptoms of the disease in observed patients with three different genetic variants of MED occurred at the age of 2–3 years old and were characterized by gait disturbance and climbing stairs difficulties. Gradually, these symptoms were accompanied by pain in large joints. According to the ultrasound examination of the joints, signs of synovitis were noted, as a result they were diagnosed with JIA (polyarticular variant, seronegative for rheumatoid and antinuclear factor) and immunosuppressive therapy were prescribed without significant effect. The atypical course of the JIA was the reason for additional examination of patients by an orthopedist and geneticist. Careful analysis of the large joints radiographs made it possible to suspect one of the variants of MED in our patients based on the detection of distinctive signs, which were characterized by abnormal ossification (diminished size and flattening) of the epiphyses and abnormal shape and structure of the femoral head epiphysis. Molecular genetic analysis was performed to confirm the diagnosis. As a result, a pathogenic variant of the nucleotide sequence in the COMP gene was detected in one of the patients, two pathogenic variants in the SLC26A2 gene in another patient, and one pathogenic variant in the COL9A2 gene in the third patient, which made it possible to confirm the final diagnosis of MED type 1 with an autosomal dominant type of inheritance, MED type 4 with an autosomal recessive type of inheritance and MED type 2 with an autosomal dominant type of inheritance, respectively. Based on the results of our own research and analysis of the literature data, key directions for the differential diagnosis of MED and JIA were formulated. It is shown that the analysis of the X-ray images of patients is essential in differential diagnosis.
Conclusion. Despite the significant overlap of the clinical symptoms between JIA and MED, the key to the early diagnosis of MED is a comprehensive examination, which included genealogical analysis, features of clinical manifestations and disease course in combination with distinctive radiological signs including delayed ossification of the epiphyses of tubular bones typical for MED. However, the question remains about the probability of a combined nature of osteoarticular disorders, i. e., the possible development of JIA in patients with hereditary skeletal dysplasias which requires in-depth study in the future.

Coxitis belongs to the extraaxial manifestations of ankylosing spondylitis (AS) and is considered as an unfavorable prognostic factor that may play a significant role in the development of functional limitations.
The aim of the study – to evaluate the functional status of the hip joints (HJ) before total hip replacement (THR) in AS patients.
Materials and methods. The retrospective study included 170 patients with AS who met the modified New York criteria of 1984, who were hospitalized in the traumatology and orthopedics department of the V.A. Nasonova Research Institute of Rheumatology from 1998 to 2020, and all patients underwent scheduled THR. Most of them were males (80.6%). The average age of the patients was 38,1±11,3 years, duration of the disease from the moment of the first symptoms appearance – 17,0±8,5 years, duration of pain in HJ before THR – 7,4±4,8 years. Disability was established in 80% of cases: in 14,1% – group 1, in 50% – group 2, in 15,9% – group 3. Did not work due to the disease – 64% of patients. The preoperative functional state of HJ was determined using the modified Harris scale.
Results and discussion. The preoperative total Harris score averaged 38.0±15.4 points and the pain score averaged 15.6±8.4 points. In the vast majority (82.9%) of patients it ranged from 10–20 points (“severe” and “moderate constantly”). The intensity of pain according to VAS was 72,3±14,0 mm. The median severity of lameness in the HJ was 5 [0; 5] points. Claudication was absent in 2.4% of patients, moderate in 42.9% of cases, severe in another 42.9%, and mild in 11.8%. Additional support was needed in the majority of patients (69.4%). One (29.4%) or two (26.5%) crutches were more frequently required. There was no need for additional support in 30.6% of patients. In 68.8% of cases, there were varying degrees of limitation in distance walking. Climbing stairs caused difficulties in 167 (98.2%) patients, only 3 (1.8%) of them did not use a handrail. Only 1 in 4 patients could use public transportation. Various anatomical deformities were determined in 88.8% of cases. Almost all patients were found to have pronounced restrictions in the volume of movements in the HJ.
Conclusion. The result of function assessment according to the Harris scale, as well as the presence of deformities and significant limitation of the range of motion indicate a high degree of severity of the HJ lesion in patients with AS before THR.

The aim of this article was an attempt to answer the question: is necrotizing sarcoid granulomatosis a late stage of nodular sarcoidosis or an independent disease? In order to better understand the designated topic, as part of the description of the clinical case, a differential diagnosis of a number of granulomatous diseases with vasculitis was carried out. The main emphasis was placed on the accurate interpretation of pathomorphological features in each individual case.