The relevance of study systemic connective tissue diseases with juvenile onset for modern medicine is determined by the trend towards their growth in the population, the difficulty of early diagnosis, the rapid development of disability and a poor life prognosis. The article presents the main achievements in the study of this group of diseases in children, with an emphasis on the most significant issues from a practical point of view related to diagnosis, classification, clinical features and modern approaches to treatment.

This article presents review of literature of the history of international classification criteria of American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) 2016, considers the features of validation and development of preliminary European criteria 1993, criteria of American European Consensus Group (AECG) 2002 and ACR 2012 criteria.

Interleukin (IL) 6 is one of the key cytokines whose role in the inflammation development in rheumatoid arthritis (RA), is well proven. The pleiotropic effects of the cytokine and biologic agents that inhibit its action have been studied much worse. The review provides information on the effects of IL-6 and blocking its signaling pathway on adipose tissue, glucose metabolism and adipocytokine levels in RA. It has been shown that prolonged blockade of IL-6 receptors does not lead to the adipose tissue accumulation and improves glycemic control, although it is not clear whether such effect is associated only with the anti-inflammatory properties of tocilizumab and sarilumab. Moreover, the mechanism of this beneficial effect is not fully understood, since the data on increased sensitivity of peripheral tissues to insulin during tocilizumab treatment are ambiguous. Perhaps changes in the relationship of adipocytokines or hormones play a certain role.

The medical and social significance of systemic sclerosis (SSc) is high. The progressive disease has a significant impact on the functional status, work participation and leads to early disability in patients of working age. The article presents data on the prevalence of work disability in patients with SSc in comparison with other rheumatic diseases, the frequency of separation from work and work transitions due to the problems connected with SSc, the socio-economic burden of SSc in different countries. The article specifies the components of the disease which affect the ability to work, the main approaches to quantify the indicators of working ability, describes the instruments most commonly used for this purpose. The data of various authors on working ability measurement and predictors of work disability in patients with SSc are presented.

The term of “incomplete” systemic lupus erythematosus (iSLE) is used when patients have typical clinical and immunological signs of lupus without fulfilling the classification criteria for SLE. Autoantibodies appear in patients years before diagnosis, and the most common clinical manifestations are nonspecific and may be the only symptom of the disease for some time. Progression to definite SLE occurs in 5–57% of patients with iSLE within 1–10 years. There are currently no recommendations for monitoring and treatment of iSLE patients. This article presents the results of our own research and literature analysis on clinical and pathogenetic problems of iSLE.

Idiopathic inflammatory myopathies (IIM) are a group of chronic autoimmune conditions characterized by proximal muscle weakness and potentially accompanied by a range of extramuscular clinical manifestations. There are subtypes of IIM including dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myopathy (IMNM), sporadic inclusion body myositis (sIBM), overlap myositis (OM) with subgroup of anti-synthetase syndrome (ASS) and cancer-associated myositis. Taking into account rarity of the disease, heterogeneity of clinical presentation, difficulties in detection methods and interpretation of myositis associated autoantibodies (MAAs) and myositis specific autoantibodies (MSAs), search for objective imaging methods of muscle damage continues. This is important to definitive diagnosis, predicting subtypes of IIM and case follow-up. One of the most promising methods is magnetic resonance imaging (MRI). The aim of the review was to examine the role of MRI in assessment muscle damage, in particular, most typical MRI-findings and there features in different types of IIM with further clinical cases.

Background. Netakimab has shown high efficacy in controlled clinical trials in the treatment of patients with ankylosing spondylitis (AS). This article presents results of an observational study of netakimab using in routine clinical practice.

Methods. Patients were recruited for the study from August 2020 to December 2021 at 23 centers in the Russian Federation. The study included patients who were prescribed netakimab therapy before enrollment, so clinical and medical history data for the first visit were entered retrospectively, and following visits at weeks 12, 24 and 52 of therapy were collected within the study. Drug survival rate has been calculated according to Kaplan – Meier analysis.

Results. The study included 137 patients (93 men and 14 women) with AS. The average age of patients was 42.3 (±11.9) years, 34.3% of patients had previously received therapy with genetically engineered biologic drugs, mainly tumor necrosis factor inhibitors. At the end of the analyzed period (52 weeks of therapy), 90.4% [95% CI: 85.4–95.7] of patients continued treatment with netakimab. The BASDAI and ASDAS-CRP showed statistically significant decreases in scores from baseline at all time points. Netakimab was well tolerated by patients; adverse effects (AEs), related to therapy according to the investigator’s opinion, were reported in 7 (5.1%) patients. 2 patients stopped taking netakimab due to AEs: terminal ileitis and chronic colitis.

Conclusions. In real-world clinical practice, netakimab demonstrated high retention rates, a favorable safety profile, and sustained efficacy throughout the first year of therapy.

The aim – to present the experience of diagnosis, management, and therapy with IL-1 inhibitors in patients with Schnitzler’s syndrome (SchS) according to a multicenter Russian cohort.

Materials and methods. In an observational retrospective study for a 10-year period (2012–2022), 17 patients with SchS who were admitted to the hospital or were observed on an outpatient basis, among them 8 women, 9 men, were included in the study. The diagnosis of all corresponded to the Strasbourg diagnostic criteria.

Results. The age of patients ranged from 25 to 81 years (Me 53 [46; 56] years). The age at the time of the onset of the disease ranged from 20 to 72 years (Me 46 [39; 54] years), the duration of the disease before diagnosis ranged from 1 to 35 years (Me 6.5 [3; 6] years), in 3 it exceeded 10 years, in the rest it ranged from 1 to 8 years. Infectious and lymphoproliferative diseases, monogenic AIDS (CAPS, TRAPS, HIDS) were excluded from all patients at the prehospital stage. The guiding diagnosis for all was Still’s disease in adults. Clinical manifestations of the disease included: fatigue, lethargy, fatigue, rash and fever in all, skin elements were urticular in all, accompanied by itching in 6 (37.5%). Bone pain had 12 (70.6%), arthralgia – 16 (94.1%), arthritis – 9 (52.9%), myalgia – 7 (41.2%), weight loss in 4 (23.5%). Lymphadenopathy (6), enlarged liver (6), pericarditis (4), angioedema (6), redness and dryness in the eyes (3), sore throat (2), abdominal pain (1), distal polyneuropathy (2), paraesthesia (1), chondritis of the auricles were less common (1). Monoclonal gammopathy was detected in all with a secretion level of 2.9–15.1 g/l: IgMk (n=10 (64.7%)), less often IgMλ (n=2), IgGk (n=2), IgGλ (n=1), IgAλ (n=1). Ben-Jones protein was not detected in any of them. All patients had an increase in the level of ESR, CRP. 16 patients before inclusion in the study received GC (94.1%) with a temporary effect and its escape with dose reduction or cancellation, DMARD – 7, among them methotrexate (5), hydroxychloroquine (2), cyclophosphamide (1), also NSAIDs and antihistamines in all, biological drugs: anti-B-cell the drug rituximab (1), monoclonal AT to IgE – omalizumab in 2 (1 – without effect, 1 – partial effect). 11 patients were prescribed IL-1: canakinumab – 9 (52.9%) subcutaneously once every 8 weeks, anakinra – 4 (23.5%) subcutaneously daily. The duration of taking anakinra, which was prescribed in the test mode, ranged from 1 week to 2.5 months with a further switch to canakinumab in 3. The duration of taking canakinumab at the time of analysis ranged from 7 months to 8 years. Against the background of treatment with IL-1, 10 out of 11 (90.9%) received a complete response from the clinical manifestations of the disease and a decrease in the level of ESR and CRP within a few days. In 1 patient, a partial response was received to the administration of anakinra, and when switching to canakinumab, the effect of treatment was finally lost. 1 patient received IL-6 for 8 months with incomplete effect and transition to IL-1 with positive dynamics. In 1 patient, due to the persistent absence of relapses, the interval between canakinumab injections was increased to 5 months without signs of reactivation, but subsequently, against the background of stress and relapses of the disease, the intervals were reduced to 4 months. A healthy child was born in the same patient on the background of treatment. The tolerability of therapy was satisfactory in all patients, no SAE was noted.

Conclusion. SchS is a rare multifactorial/non–monogenic AID that needs to be differentiated from a number of rheumatic diseases and other AIDS. The onset in adulthood, the presence of recurrent urticarial rashes in combination with fever and other manifestations of a systemic inflammatory response are indications for examination for monoclonal secretion. The use of short- or long-acting IL-1 is a highly effective and safe option in the treatment of such patients.

The aim of the study was to identify the relationship between bone mineral density disorders and chronic obstructive pulmonary disease among residents of Bishkek, Kyrgyz Republic, taking into account risk factors.

Material and methods. 200 residents of lowland (mean age 56,9±1,7 years) were examined. Bone mineral density (BMD) was determined by dual-energy X-ray absorptiometry.

Results. Chronic obstructive pulmonary disease (COPD) was almost 2-fold more common among patients with low BMD than healthy controls (63.0% and 34.8% of cases, respectively; p<0.001), despite the same frequency of smoking. Other independent predictors of low BMD were glucocorticoid intake and age.

Conclusion. The occurence of COPD is an independent risk factor for the development of osteopenia/osteoporosis even after age and other known risk factors adjustment in lowland residents of the Kyrgyz Republic.

Aim of the study – to assess the relationship of the osteoporotic phenotype of body composition with nutritional and physical status in women with rheumatoid arthritis (RA).

Material and methods. 104 women (average age 59.5±8.7 years) with RA were enrolled. The examination included clinical, laboratory and instrumental (dual-energy X-ray absorptiometry) methods. Nutritional status was assessed using the Mini Nutrition Assessment-Short Form (MNA-SF), physical status – using the International Physical Activity Questionnaire (IPAQ), “Chair stand test”, handgrip strength, “Timed Up and Go test” and gait speed.

Results. Osteoporotic phenotype was diagnosed in 38.5% of patients. Malnutrition and risk of malnutrition according to MNA-SF had 51.0% of women. Low and moderate level of physical activity according to IPAQ – 51.9% of patients. In multivariate logistic regression analysis, independent factors associated with osteoporotic phenotype were determined: walking <30 minutes a day (odds ratio (OR) – 1.34; 95% confidence interval (95% CI): 0.11–17.32), low muscle strength of the upper extremities (handgrip strength less than 16 kg) (OR=7.12; 95% CI: 1.02–49.57) and lower extremities (“Chair stand test” more than 15 seconds) (OR=4.45; 95% CI: 1.08–18.42), body mass index (BMI) less than 25 kg/m² (OR=1.39; 95% CI: 1.04–1.85).

Conclusion. A high frequency of the osteoporotic phenotype of body composition was revealed in patients with RA, among whom almost half of the examined individuals had insufficient nutrition and/or reduced physical activity. Walking <30 minutes a day, low upper and lower limb muscle strength, and BMI<25 kg/m² were associated with the osteoporotic phenotype.

The aim of the study was to evaluate the clinical manifestations and survival of patients with giant cell arteritis (GCA).

Methods. A retrospective study included 166 patients with newly diagnosed GCA. Clinical, laboratory and instrumental data, three sets of classification criteria were used to confirm the diagnosis: the American College of Rheumatology (ACR) 1990, the revised ACR criteria of 2016 and/or the new ACR and European Alliance of Associations for Rheumatology (EULAR) 2022 criteria. Some of the patients underwent instrumental investigations: temporal artery ultrasound Doppler (n=61), contrast-enhanced computed tomography (CT) (n=5), CT angiography (n=6), magnetic resonance imaging (n=4), magnetic resonance angiography (n=3) and 18F-FDG positron emission tomography/CT (n=47). Overall and recurrence-free survival were analyzed using survival tables, Kaplan – Meier method.

Results. The most frequent first manifestations of GCA were headache (81.8%), weakness (64%), fever (63.8%) and symptoms of rheumatic polymyalgia (56.6%). Changes of temporal arteries in color duplex scanning were detected in 44 out of 61 patients. GCs therapy was performed in all patients who agreed to be treated (n=158), methotrexate was used in 49 out of 158 patients, leflunomide – in 9 patients. In 45 (28.5%) out of 158 patients a stable remission was achieved as a result of GCs monotherapy, in 120 (75.9%) patients long-term maintenance therapy with GCs was required to prevent exacerbations, including 71 (44.9%) patients – in combination with methotrexate or other immunosuppressive drugs. The follow-up period of patients with a history of relapses was 21.0 (8.0–54.0) months. Relapses developed in 73 (46.2%) patients. The overall one-year survival rate was 97.1% [95% confidence interval (CI): 94.3; 99.9], and the five-year survival rate of patients was 94.6% [95% CI: 90.2; 99.0]. The one-year relapse-free survival rate was 86.4% [95% CI: 80.5; 92.3], and the five-year relapse-free survival rate was 52.4% [95% CI: 42.0; 62.8]. 12 (7.2%) of 166 patients died. The cause of death was myocardial infarction in two patients, stroke in two patients, and breast cancer in one patient; in the remaining seven cases, the cause of death was not determined.

Conclusion. Given the high frequency of disease exacerbation, patients with GCA require long-term follow-up, especially during the first year after diagnosis.

The pathogenesis of immunoinflammatory rheumatic diseases (IRDs) is based on chronic inflammation, one of the key mechanisms of which may be abnormal activation of macrophages, leading to further disruption of the immune system.

The aim – to evaluate the pro-inflammatory activation of circulating monocytes in patients with IRDs.

Material and methods. The study included 149 participants: 53 patients with rheumatoid arthritis (RA), 45 – with systemic lupus erythematosus (SLE), 34 – with systemic scleroderma (SSc) and 17 participants without IRD, aged 30 to 65 years. Basal and lipolysaccharide (LPS)-stimulated secretion of monocytes was studied in a primary culture of monocytes obtained by immunomagnetic separation from blood. Quantitative assessment of the cytokines tumor necrosis factor-α (TNF-α), interleukin 1β (IL-1β) and the monocyte chemoattractant protein-1 (MCP-1) was carried out in the culture fluid by ELISA. Pro-inflammatory activation of monocytes was calculated as the ratio of LPS-stimulated and basal secretions.

Results. It was shown that the basal secretion of all studied cytokines was significantly increased in all groups of patients with IRDs, except for the secretion of IL-1β in the SLE group, compared with the control. LPS-stimulated secretion of TNF-α was increased and MCP-1 was decreased in patients with IRDs compared to the control group; LPS-stimulated IL-1β secretion only in the SSc group was significantly different from the control group. In the RA group, monocyte activation was reduced for all cytokines compared to the control, in the SLE group – for TNF-α and MCP-1, in the SSc group – for MCP-1.

Conclusion. The decrease in pro-inflammatory activation of monocytes in patients with IRDs is due to a high level of basal secretion of cytokines, which can lead to disruption of the adequate immune response in these diseases and is an important link in the pathogenesis of chronic inflammation.

Objective. Assess the level of antibodies to carbamylated proteins (anti-CarP) and analyse the clinical and immunological associations in patients with ACCP-negative and ACCP-positive variants of rheumatoid arthritis.

Materials and methods. 150 patients with a reliable diagnosis of rheumatoid arthritis and 25 patients as healthy controls were included in the study. Depending on ACCP values, two groups of patients were recruited: ACCP-positive (n=75) and ACCP-negative (n=75). RA activity was assessed by the DAS28 (Disease Activity Score 28) index. Determination of antibodies to carbamylated proteins was performed by enzyme-linked immunosorbent assay (BlueGene Biotech, China). Quantitative determination of ACCP in serum was performed by enzyme immunoassay using a commercial reagent kit (AxisShield, UK; upper limit of normal 5.0 U/ml; Orgentec, Germany; upper limit of normal 20.0 U/ml).

Results and discussion. Me for anti-CarP in patients with RA was 126.2 [100.83; 157.41] ng/ml and was statistically significantly higher (p<0.001) than healthy controls 88.89 [70.53; 107.75] ng/ml. Among all patients with RA, 50 (33.3%) were anti-Carp positive, 22 (29.3%) were anti-Carp(+) in the ACCP(+) group, 28 (37.3%) in the ACCP(–) group, and 1 (2%) volunteer from healthy controls (p=0.002). In ROC analysis to assess the diagnostic significance of anti-Carp for RA for all patients with RA, the area under the curve was 0.783±0.047 (95% CI: 0.691–0.874; p<0.001), with a cut-off point of 143 ng/ml, specificity 96%, sensitivity 36.7%.

In the ACCP(+) RA group, the erosion count was statistically significantly higher (p=0.044) in anti-CarP(+) patients than in anti-CarP(–) patients. A weak direct correlation between anti-CarP and DAS28 was found in the ACCP(–) RA group.

Conclusion. We studied the predictive value of anti-CarP as an adjuvant biomarker in ACCP(+) and ACCP(–) subtypes of RA. ACCP(+), anti-CarP(+) patients have a more “erosive” subtype of the disease than ACCP(+), anti-CarP(–) patients. In ACCP(+) patients, anti-CarP helps to identify a more erosive subtype of the disease, and among ACCP(–) patients, it helps to reduce the proportion of seronegative patients. Further studies are needed to determine the optimal standards for the laboratory diagnosis of anti-CarP and to clarify the diagnostic potential of these antibodies as part of the differential diagnosis of arthritis in other rheumatic diseases.

Introduction. Hip-spine syndrome (HSS) is a combination of coexisting hip osteoarthritis (OA) and degenerative lumbar spine stenosis (LSS). Main difficulties in treating patients with HSS are in early diagnostics and in choosing right surgery, because mistakes lead to pain maintenance. Existing diagnostic algorithms show right surgery choosing failure in 15–20%. We present results of examination patients with HSS in our survey.

The aim – to present clinical and instrumental results of examination of patients with hip-spine syndrome.

Materials and methods. We have examined 378 patients with typical pain pattern (buttocks, low back spine, groin and lateral hip) and difficulties in pain source definition. We performed hip X-rays and low back spine MRI.

Due to results we divided patients into three groups – patients with HSS (n=100), with hip OA (n=172) and patients with LSS (n=106). We used Harris Hip Score, Oswestry Disability Index and Visual Analogue Score to determine hip and lumbar spine functional status and pain level.

Results. Patients with HSS had higher (p<0.05) pain levels (76.5±9.1 mm) than patients with hip (68.3±7.9 mm) or lumbar spine pathology (67.4±7.9 mm). Harris Hip Score in patients wirh HSS (52.7±8.1 points) was same as in patients with hip OA (55.5±9.1 points), Oswestry Disability Index in these patients (44.2±7.6%) was same as in patients with LSS (43.2±7.8%).

Conclusion. High pain level and low Harris Hip Score and Oswestry index, along with clinical examination, on first visit can help suspect hip-spine syndrome and recommend both hip and lumbar spine imaging.

Gout is the most common chronic autoinflammatory disease, the development of which is associated with persistent hyperuricemia caused by both environmental and genetic factors, which leads to the deposition of sodium monourate crystals in various tissues and organs of the human body. Gout is more common in men than in women of childbearing age, due to the uricosuric effect of estrogen, however, after menopause, the incidence of gout in women increases significantly. At the onset of the disease, the first metatarsophalangeal joint, ankle and knee joints are most often involved in the pathological process. However, there are isolated reports in the literature about a rare gout lesion of the axial skeleton, for example, the sacroiliac joint, in which the nature of the pain syndrome, magnetic resonance imaging, and X-ray picture can mimic spondyloarthritis. The article presents a rare case of damage to the axial skeleton in a 57-year-old patient with gout, manifested by acute inflammatory back pain and arthritis of the lower extremities.