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Currently, the prognosis for systemic lupus erythematosus (SLE) has improved significantly, but the relative risk of death in these patients is stillhigher than in the general population. Thrombotic complications are one of the leading causes of death in SLE. Objective: to analyze the survival rate and structure of lethal outcomes in Orenburg population of patients with SLE, including deaths due to thrombotic complications. Material and methods. A two-stage study of SLE progression and patient survival was conducted from 2007 to 2022. Clinical signs of the diseasewere analyzed in all patients at baseline (n = 68) and in survivors (n = 50) after 15 years. The median age at the time of enrolment in the study was 35 [29; 45] years, the disease duration – 7.5 [3; 13.5] years. During the second stage, the characteristics of the course of the disease in the survived patients and the causes of death in those who died over 15-year period were determined. Results and discussion. The 10-, 15- and 20-year survival rates in Orenburg population of patients with SLE reached 98.5, 95.5 and 86.3%, respectively. During this period, 18 (26.5 %) deaths were registered, the median age of the deceased was 48.5 [39; 57] years, and the duration of the disease was 22 [16; 30] years. The most common causes of death were thrombotic complications (n = 14, 78 %) due to antiphospholipid syndrome, lupus nephritis, and arterial hypertension. Less frequently, infectious complications were the cause of death (n = 4, 22 %). Patients with thrombotic complications had a 20-year survival rate of 80.2% that was significantly lower than in the SLE group without thrombosis. Conclusion. The results obtained allow to consider the presence of thrombotic complications in patients with SLE in Orenburg population as an unfavorable prognostic factor.

NiSpAR is a non-interventional, multicenter study whose aim was to describe a cohort of patients with non-radiological axial spondyloarthritis (nr-axSpA) and approaches to its diagnosis in the Russian Federation.Material and methods. The study involved 20 research centers in different regions of the Russian Federation. The work consisted of two phases: retrospective data collection 12 months before enrolment in the study and prospective observation of patients in whom the diagnosis of nr-axSpA was confirmed (104 weeks).The study included 272 patients who met the inclusion and exclusion criteria (Full Analysis Set, FAS). The mean age of the FAS-population was 38.7±11.0 years. The diagnosis of nr-axSpA was confirmed in 159 (58.5 %) of the 272 patients. Of the remaining 113 (41.5%) participants, 57 (50.4%) did not have the information required to confirm the diagnosis, 34 (30.1%) did not have pelvic radiographs, and 22 (19.5%) did not fulfil the ASAS criteria for nr-axSpA.Results and discussion. The mean age of patients with nr-axSpA was 37.6±10.4 years, more than half of them (52.8%) were women. The median disease duration was 36 [12; 80] months. In half of the patients the disease duration was more than 2 years, in more than one third – less than 2 years. The median CRP and ESR values were 5.0 [2.0; 12.0] mg/l and 11.0 [5.0; 18.0] mm/h, respectively. The BASDAI averaged 3.5±2.0 and was >4 in more than half of the cases (64.2%). The mean ASDAS-CRP value reached 2.6±1.1. Only 1 in 5 participants (20.8%) had low axSpA activity, while in 61.1% it was high (44.7%) or very high (16.4%). An inactive state was found in a small number of patients (9.4%). Twenty two (13.8%) patients had treatment with biologic disease-modifying antirheumatic drugs (bDMARDs) in anamnesis, and 21 (13.2%) patients were still taking them.Conclusion. The results of the retrospective phase of the study show that in real-life clinical practice in the Russian Federation there is a continued positive trend towards improving the diagnosis of nr-axSpA. The median duration of the disease at the time of enrolment in the study was 3 years. The frequency of use of magnetic resonance imaging has increased by more than 1.8 times. At the same time, practitioners still have difficulties in establishing the diagnosis of nr-axSpA and a shortage of bDMARDs for treatment of these patients.

The mechanism of osteoporosis (OP) development in systemic sclerosis (SSc) remains unclear.Objective: to assess bone mineral density (BMD) and the level of bone metabolism markers (osteocalcin — OC, — C-terminal type I collagen telopeptides — b-CrossLaps) in the blood serum of patients with SSc.Patients and methods. 65 patients with SSc were examined, 6 (9%) men and 59 (91%) women, the average age was 51 [39; 61] year (main group), and 35 healthy individuals comparable in anthropometric parameters (control group). In all individuals were assessed the most important populational risk factors for OP. BMD was determined using dual energy X-ray absorptiometry (DXA); the level of vitamin D, OC and b-CrossLaps in blood serum — by enzyme immunoassay.Results and discussion. A decrease in BMD was statistically significantly more common in patients with SSc (46, 71%), than in controls (11, 31%). Significant risk factors for OP in SSc were early menopause, low physical activity, hypovitaminosis D, and probably high activity and duration of the disease. In patients with SSc, there was a significant decrease in the level of OC compared with the controls; in patients with a reduced BMD, the content of OC was significantly less than in patients with normal BMD. The average values of b-CrossLaps in the main and control groups were comparable, but in patients with OP this parameter was lower than in those with normal BMD.Conclusion. In patients with SSc, OP develops statistically significantly more often than in healthy individuals. Risk factors for OP are early menopause, low physical activity, long duration and high activity of SSc. The predominance of bone formation impairment over bone resorption as a mechanism for the development of secondary OP was noted.

Currently, the causes of extra-axial and extra-skeletal manifestations of ankylosing spondylitis (AS) and the possible impact of genetic aspects on its course and clinical features remain unresolved.Objective: to investigate the association of the polymorphic markers rs10050860 and rs17482078 in the ERAP1 gene and rs11209026 in the IL23R gene with the development and clinical manifestations of AS.Patients and methods. An allele-specific polymerase chain reaction assay was carried out to assess the alleles and corresponding genotypes of ERAP1 and IL23R gene polymorphisms in 70 patients (49 men and 21 women; mean age, 38 [31; 49] years) with AS and in 20 healthy donors. The activity indices, ESR, CRP, and extra-axial and extra-skeletal manifestations of AS were assessed in patients at the time of the investigation and in their history.Results and discussion. The results of genotyping showed a significant association of the studied markers with AS. The carriage of the C/T genotype of the polymorphic markers rs10050860 and rs17482078 in the ERAP1 gene was associated with the history of peripheral arthritis (p=0.029) and the presence of incomplete right bundle branch block (IRBBB) (p=0.003 and p=0.006); the carriage of the G/A genotype of the marker rs11209026 in the IL23R gene was significantly associated with psoriasis (p=0.017) and IRBBB (p=0.03) in patients with AS.Conclusion. The polymorphic markers of the ERAP1 and IL23R genes are associated with the risk of developing AS in this sample of patients. There is a significant correlation between the studied polymorphisms and some clinical manifestations of AS, which can be considered as a predictor of a more severe disease course.

The role of trace elements (TEs) and their imbalance in the physiology of bone tissue and in the development of inflammatory diseases of the joints and spine has been discussed in recent years; however, there is no evidence for the TE status of patients with ankylosing spondylitis (AS) and its possible impact on the course of the disease.Objective: to investigate the impact of the TE status of patients with AS on the course, clinical manifestations, and activity of the disease.Patients and methods. Examinations were made in 58 patients (39 men and 19 women), residents of the Orenburg Region, with a reliable diagnosis of AS, the duration of which was 16 [11; 26] years. The patients’ mean age was 38 [31; 48] years. HLA-B27 antigen was detected in 91.4% of cases. In addition to the generally accepted examination, atomic absorption spectrophotometry was used to determine the hair levels of 9 TEs: Cu, Zn, Fe, Mn, Cr, Co, Ni, Pb, and Cd in all the patients.Results and discussion. The AS patients living in the Orenburg Region showed TE imbalance manifested by Cu and Zn deficiency and Ni, Cr, and Mn accumulation in the hair. Multidirectional correlations were found between the values of these TEs and the presence of extra-axial (peripheral arthritis, dactylitis) and extra-skeletal (uveitis) manifestations of AS, its activity, and severity of functional disorders.Conclusion. The preliminary results may suggest that the emerging imbalance of TEs can affect the course of AS, maintaining and increasing its activity.

The mechanism of development of osteoporosis (OP) in systemic scleroderma (SSc), despite ongoing studies on this subject, remains not completely clear.Objective. To assess the state of bone mineral density (BMD) in patients with systemic scleroderma (SSc) and to determine the level of bone metabolism markers (osteocalcin, b-CrossLaps) in blood serum.Methodology. We examined 65 patients with SSc: 6 (9%) men and 59 (91%) women, with an average age of 51 [39;61] years and 35 relatively healthy individuals comparable in basic anthropometric parameters with patients of the main group. The main population risk factors for osteoporosis were assessed. Bone mineral density (BMD) was assessed using dual energy X-ray absorptiometry (DXA). The level of vitamin D, osteocalcin (OC), and b-CrossLaps in blood serum was determined by enzyme immunoassay.Results. A decrease in BMD was significantly more often observed in patients with SSc - 46 (71%) than in controls - 11 (31%). Significant risk factors for OP in SSc were the presence of menopause that occurred at an early age, low physical activity, hypovitaminosis D, probably high activity and duration of the disease. In patients with SSc, there was a significant decrease in the level of OK compared with the control; in the presence of a decrease in the BMD, the content of OK was significantly lower than with a normal BMD. Mean values ​​of b-CrossLaps in SSc were comparable with the control; in patients with OP, the parameter was lower than in those with normal BMD.Conclusion. OP develops significantly more often in SSc compared to the control. Risk factors for OP are early menopause, low physical activity, duration and activity of SSc. The predominance of bone formation disorders to a greater extent than bone resorption as a mechanism for the development of secondary OP was noted.

The paper gives the definition of remission in axial spondyloarthritides (axSpA), which has been developed by the Spondyloarthritis Study Group of Experts. The work used the Delphi technique. At stage 1, based on the analysis of the data available in the literature and on their own clinical experiences, the experts proposed some variants of the definition of remission and ways of its evaluation in patients with axSpA. At Stage 2, the definitions that had received at least 80% of the votes via anonymous voting were selected and adopted without further discussion. Those that had received an equal number of votes were reconsidered and additionally discussed; then there was repeat voting, by choosing the final definition. As a result of their discussion, the experts formulated the definitions of clinical laboratory and magnetic resonance imaging (MRI) remissions. They proposed the following remissions in axSpA: drug and drug-free, clinical laboratory, and MRI remissions, as well as a remission in the presence and absence of structural changes in the locomotor system, as evidenced by imaging techniques. Criteria for clinical laboratory remission and basic tools for its evaluation that can be used in real clinical practice and researches have been elaborated.

The paper gives recommendations for the drug therapy of axial spondyloarthritides, which have been developed by the Spondyloarthritis Study Group of Experts. The recommendations describe the patient management tactic in the most common clinical situations, which is aimed at maximizing the efficacy and safety of treatment.

The paper gives the recommendations for the assessment of disease activity and functional status in patients with ankylosing spondylitis in clinical practice, which have been developed by experts, by taking into account international and Russian experience in managing these patients.

The purpose of the research was studying of features of clinic, course, medical tactics and outcomes of infectious endocarditis in 25 patients suffering from “salt” drug addiction. Materials and methods: we examined 25 patients with infectious endocarditis, the host “salt” intravenously. To verify the diagnosis in addition to standard studies of blood, urine, sputum, was performed transthoracic EchoCG, x-ray study of the chest (twice, at admission and before discharge of the patient from the hospital). For certain indications used diagnostic fiber-optic bronchoscopy and computed tomography of the lungs, consulted a neurologist, cardiologist, infectious disease. Results and discussion: 84% of patients diagnosed with HIV. The acute course of the disease and presence of accompanying pathology of organs and systems was observed in the majority of patients, with a predominance of neurological changes (88%). Typical meager cardiac symptoms (noise in the region of the heart were heard in 12% of patients), frequent lung as a result of pulmonary embolism (64%). Identified infectious agents (in most cases Staphylococcus aureus -32%), the defeat of the heart valves according to the EchoCG (in 72% of patients vegetation on tricuspid valve with varying degrees of regurgitation). The choice of antimicrobial medicines was optimized (cephalosporins I-III generation, aminoglycosides, vancomycin). The positive outcome of a disease was observed in 84% of patients, mortality was 16%. Conclusions: for infectious endocarditis injecting a “salt” of the drug revealed the following: acute disease; pronounced General symptoms, atypical clinical picture, involvement of the heart valves, lungs result in pulmonary embolism, early development of multiple organ failure, high mortality. In comparison with injection drug users who inject other drugs, “salt” addicts are characterized by the frequent development of infectious endocarditis, which is apparently connected with the influence of “salt” on the immune system and joining the bright and varied neurological symptoms.

The European Society for Clinical and Economic Aspects of Osteoporosis and Osteoarthritis (ESCEO) treatment algorithm for the management of knee osteoarthritis (OA), published in December 2014, provides practical guidance for the prioritization of interventions. This current paper represents an assessment and endorsement of the algorithm by Russian experts in OA for use in Russian clinical practice, with the aim of providing easy-to-follow advice on how to establish a treatment flow in patients with knee OA, in support of the clinicians’ individualized assessment of the patient. Medications recommended by the ESCEO algorithm are available in Russia. In step 1, background maintenance therapy with symptomatic slow-acting drugs for osteoarthritis (SYSADOA) is advised, for which high-quality evidence is provided only for the formulations of patented crystalline glucosamine sulphate (pCGS) (Rottapharm/Meda) and prescription chondroitin sulfate. Paracetamol may be added for rescue analgesia only, due to limited efficacy and increasing safety signals. Topical non-steroidal anti-inflammatory drugs (NSAIDs) may provide additional symptomatic treatment with the same degree of efficacy as oral NSAIDs but without the systemic safety concerns. To be effective, topical NSAIDs must have high bioavailability, and among NSAIDs molecules like etofenamate have high absorption and bioavailability alongside evidence for accumulation in synovial tissues. Oral NSAIDs maintain a central role in step 2 advanced management of persistent symptoms. However, oral NSAIDs are highly heterogeneous in terms of gastrointestinal and cardiovascular safety profile, and patient stratification with careful treatment selection is advocated to maximize the risk: benefit ratio. Intra-articular hyaluronic acid as a next step provides sustained clinical benefit with effects lasting up to 6 months after a short-course of weekly injections. As a last step before surgery, the slow titration of sustained-release tramadol, a weak opioid, affords sustained analgesia with improved tolerability.

The paper gives the draft guidelines elaborated by the Expert Spondyloarthritis Diagnosis and Treatment Group by order of the Association of Rheumatologists of Russia. The guidelines include the essentials of how to use nonsteroidal anti-inflammatory drugs in axial spondyloarthrititides, including ankylosing spondylitis, contain instructions for how long they should be administered, and describe possible patient management tactics in the most common clinical situations and a preferential algorithm for evaluating the efficiency and safety of treatment.

The paper provides guidelines for the use of tumor necrosis factor-α (TNF-α) inhibitors in the treatment of patients with axial spondyloarthritis (axSpA), including ankylosing spondylitis. It gives data on the efficacy of TNF-α inhibitors in patients with non-radiographic axSpA. By using international and Russian guidelines, the authors lay down indications for this therapy and criteria for evaluation of its efficiency and safety.

By the end of the first decade of the 21st century, spondyloarthritis studies have accumulated a certain number of terms that are obsolete, but used by physicians in their everyday speech, on the one hand, and a great variety of different definitions, on the other hand. In January 2014, the first organizational meeting of the Expert Group on Spondyloarthritis, Association of Rheumatologists of Russia, decided that its primary task should be to order the terminology used in this area. The authors primarily collected the terms, which had been already used in medical vocabulary, and then divided them into two categories: obsolete definitions and terms to be finalized and unified. This publication gives guidelines for using the medical terms relevant to spondyloarthritis and separately discusses how to correctly write the term sacroiliitis.

Osteoporosis in ankylosing spondylitis (AS) may exacerbate pain and functional disorders and increases the risk of fractures. The mechanisms of its development in AS have not been adequately studied.Objective: to study bone mineral density (BMD) and its regulation in patients with AS.Subjects and methods. 70 patients (mean age, 43.2±9.2 years) with a documented diagnosis of AS (mean disease duration, 17.1±7.8 years) and a control group of 30 healthy individuals were examined. All the patients underwent estimation of BMD and the serum concentrations of osteocalcin, CrossLaps, and key regulators of osteoclastogenesis, such as osteoprotegerin (OPG) and a receptor activator of nuclear factor kappa-B ligand (RANKL) by an enzyme immunoassay. Results and discussion. In patients with AS, bone metabolism was characterized by a decrease in bone formation and by some increase in bone tissue degradation especially in high AS activity. These patients showed the elevated levels of the major blocker of osteoclastogenesis OPG and the OPG/RANKL ratio, which can cause the process of ossification characteristic of AS.

62 pts with early rheumatoid arthritis (RA) were included. Neopterin changes dependence on RA clinical course and duration, acute phase markers (ESR, CRP) changes, presence of IgG and IgM rheumatoid factors, autoantibodies against cyclic citrullinated peptide as well as neopterin prognostic significance were evaluated. Immuno-inflammatory process presentation in RA was most often accompanied by elevation of neopterin serum concentration associated with more severe clinical course of early RA, significant antibody formation and radiological progression. These data can prove prognostic significance and utility of neopterin evaluation in early RA.

Aim to identify outdated terms and make changes to the terminology of spondyloarthritis. Materials and methods. At the first stage of the work, the terms divided into two categories: “outdated” definitions and terms that need to be improved or unified. Subsequently, each member of the Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia (ExSpA) presented by its own definition of the designated term or agreed with the previous term. At the next stage, the existing definitions were put together. After discussion, experts left a term that scored at least 2/3 of the votes. The special opinion of experts was recorded, whose did not coincide with the majority opinion. An open vote was conducted, when defining an “outdated” term with the unanimous decision of all group members, this term was not recommended for further clinical use. Results. The work carried out allowed us to identify a number of terms that are not recommended for use in clinical practice. Number of terms are defined, which should be used when discussing the problem of spondyloarthritis. Conclusion. The Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia suggests using or, accordingly, not using a number of terms and their definitions in clinical practice.

Objective: To define the specific features of bone metabolism and to estimate bone mineral density (BMD) in patients with early rheumatoid arthritis (ERA). Subjects and methods. Fifty-three patients (9 males and 44 females) with ERA were examined. Markers of bone metabolism markers were assessed, by measuring the serum levels of osteocalcin and C-terminal telopeptides (bCrosslaps) by electrochemoluminescence immunoassay and those of type II interleukin (IL) 1 receptor (IL1RII) and IL-6 by enzyme-linked immunosorbent assay. Bone mineral density (BMD) was studied by distal forearm densitometry (dual-energy X-ray absorptiometry) on a DexaScan DX-10 apparatus. BMD was estimated in postmenopausal women by the T-test and in men and premenopausal women by the Z-test, as well as by the absolute value of BMD (g/cm2). Results. In patients with ERA, the level of bCrosslaps was 0.50.3 ng/ml (p0.05), which is accordingly higher and lower than the reference values. There were no changes in these parameters in relation to serogroup and Xray disease stage. With higher ERA activity, there was a tendency for bCrosslaps to increase and osteocalcin to remain unchanged. There was a tendency towards decreased IL1 RII and increased proinflammatory IL-6 levels. Correlation analysis revealed a correlation between the value of BMD and the content of bCrosslaps and osteocalcin in patients with ERA and between the levels of IL-6 and bCrosslaps. Regression analysis showed a significant impact of the parameters of the course of the disease and immunological parameters on BMD changes and bone metabolism markers. Age and body mass index also affected the parameters of BMD in patients with ERA.

Objective. To study vasoregulatory function of vascular wall in pts with systemic sclerosis (SS) and chronic scleroderma-related renal disease (SRD) and determine relationship with main measures of kidney functional state. Material and methods. 25 pts with SS (24 female) with mean age 46,4±10,7 years and mean disease duration 7,5±4,7 years were included. 24 pts (96%) had diffuse form and 17 pts (68%) – generalized stage of SS. 22 pts (88%) had chronic course of the disease with typical damage of vessels, heart, lungs and skin. SRD was revealed in 17 pts (68%). Sonographic method of Celermejer D. et al. (1992) was used for assessment of vascular endothelial function. 15 healthy persons without SS were included in control group. Results. Most prominent changes of brachial artery reactivity with insufficient vasodilatation and paradoxical vasoconstriction to reactive hyperemia were revealed in most pts with moderate and severe SRD. Conclusion. These changes of endothelium dependent and endothelium independent parameters in pts with SRD pointed to irreversible probably scleroderma-related vascular wall damage.