Granulomatosis with polyangiitis (GPA) is a primary vasculitis associated with antineutrophil cytoplasmic antibodies, characterized by necrotizing vasculitis with predominant involvement of small vessels of various localizations and necrotizing granulomatous inflammation with multiple clinical manifestations. GPA remains one of the most severe systemic vasculitis with unfavorable prognosis. When analyzing the course of the disease, there are two variants of GPA, local (with lesions of the upper respiratory tract, URT, organs of vision and hearing) and generalized (with lesions of the URT, organs of vision and hearing in combination with the lungs and/or kidneys, gastrointestinal tract, nervous systems, skin involvement).
The article discusses the differential diagnosis of the disease with the nasal cavity and paranasal sinuses lesions onset, which requires an interdisciplinary approach and interaction of doctors of different specialties.

Ankylosing spondylitis (AS) and psoriatic arthritis (PsA) are the two most common diseases from the group of spondyloarthritis (SpA), which are often accompanied by permanent disability. Maintaining an acceptable quality of life, preventing the development and progression of structural changes in the musculoskeletal system, maintaining/normalizing functional and social activity are the main goals of their therapy. Over the past decades, the introduction of biological disease modifying antirheumatic drugs has made it possible to achieve significant success in the sustainable control of AS and PsA activity. However, the high cost of treatment significantly limits access to innovative drugs. Specifics of drug supply call for prescription of these drugs in the presence of the status of "disabled", which is assigned on the basis of the results of the medical and social examination (MSE). Execution of the necessary medical documentation for the implementation of the MSE is one of the functions of a rheumatologist.
The lecture analyzes the main regulatory legal acts of the Russian Federation regulating the issues of establishing disability and discusses peculiarities of medical reports processing when referring patients with AS and PsA to the MSE.

Proliferation and hyperactivation of B-lymphocytes in the salivary glands is a feature of primary Sjцgren's syndrome (pSS). Detection in saliva of proteins synthesized by B-lymphocytes may be important in the diagnosis of this disease.
Objective: to evaluate the diagnostic value of measuring the concentration of immunoglobulin free light chains (FLC) in saliva in patients with pSS.
Material and methods. The cross-sectional study included 24 patients with pSS over the age of 18 years. PSS was diagnosed according to the 2016 ACR/EULAR classification criteria. The control group consisted of 11 healthy volunteers. Blood-salivary glands histohematic barrier permeability ratio for albumin, FLC was measured. Quantitative determination of FLC and in blood and saliva was performed by enzyme immunoassay. An immunohistochemical study of biopsies of minor salivary glands (MSG) was carried out with a quantitative assessment of CD3+, CD4+, CD8+, CD20+, CD21+, CD68+, CD138+ cells. The Mann–Whitney U-test was used to compare quantitative traits. Identification of diagnostic thresholds for the concentration of FLC in saliva for the diagnosis of pSS was carried out using the ROC analysis method. An operating characteristic curve was plotted, the area under the curve, indicators of diagnostic specificity, diagnostic sensitivity, and diagnostic accuracy were calculated.
Results and discussion. The obtained values corresponded to the low permeability of the histohematic barrier of the salivary glands for albumin and FLC in patients with pSS and healthy individuals. The median concentrations of FLC ê and ë in the saliva of patients with pSS and healthy volunteers were 1.08 [0.58; 1.91], 1.038 [0.55; 2.03] mg/l and 0.36 [0.32; 0.54], 0.35 [0.21; 0.52] mg/l, respectively. The concentration of FLC in the saliva of patients with pSS was statistically significantly higher than in the control group (p<0.01). The amount of FLC ê and ë in saliva correlated with the rate of unstimulated saliva flow: rs=-0.483 (p=0.02), rs=-0.491 (p=0.017), respectively.
A relationship was found between the concentration of ê-chains in saliva and the specific number of CD138+ cells: rs=0.733 (p=0.025). Statistically significant correlations between the concentration of ë-chains and the number of mononuclear cells in the MSG have not been established.
Based on the results of ROC analysis, diagnostic thresholds for FLC concentrations in the saliva of patients with pSS were determined. Concentrations of ê- and ë-type FLC in saliva of 0.56 and 0.68 mg/l correspond to area under the curve values of 0.84 (95% confidence interval, CI 0.69–0.98) and 0.83 (95% CI 0.71–0.97), sensitivity 79.2% (95% CI 59.5–90.8) and 75% (95% CI 55.1–88), specificity 81.8% (95% CI 52.3–96.8) and 90.9% (95% CI 62.3–99.5), respectively.
Salivary FLC concentrations were compared in patients with pSS receiving and not receiving glucocorticoids (GC). The groups did not differ in a statistically significant way in terms of clinical and laboratory parameters. The median daily dose of GC was 10 [5; 10] mg in prednisolone equivalent. There were no significant differences between the concentrations of saliva FLC in patients of these groups.
Conclusion. Salivary-fixed FLCs are most likely produced by cells localized in the stroma of the salivary glands. Determination of the concentration of FLC in saliva can be proposed as a diagnostic test for the pSS. The concentration of free ê-chains in saliva can be considered as a surrogate marker of benign B-cell proliferation in the MSG. Therapy with low and medium doses of GC in pSS does not affect the concentration of FLC in saliva.

Stratification of patients into groups of high and low risk of adverse outcome is necessary for timely and early prevention of the disease, as well as the selection of adequate therapy.
Objective: to validate the global risk scale for the development of clinical manifestations of antiphospholipid syndrome (GAPSS) in a cohort of patients with primary antiphospholipid syndrome (PAPS).
Material and methods. The study included 64 patients with PAPS. Data on clinical manifestations, traditional cardiovascular risk factors, and antiphospholipid antibody profile were collected. GAPSS values were calculated for each patient by summing the scores corresponding to risk factors as follows: 3 points – for hyperlipidemia; 1 point – for arterial hypertension; 5 points – for antibodies to cardiolipin (aCL) IgG/IgM; 4 points – for antibodies to â2-glycoprotein 1 (anti-â2GP1) IgG/IgM and 3 points – for antibodies to the phosphatidylserine-prothrombin complex (aPS/PT) IgG/IgM.
Results and discussion. GAPSS indicators were comparable in women and men with PAPS – 12.0 [9.0; 13.0] points. GAPSS values did not differ in patients with thrombosis and obstetric pathology: in thrombosis they were 10.0±4.46 (range 0.0–14.0) points, in obstetric pathology – 9.26±5.08 (range 0.0–14.0) points.
The localization of thrombosis did not affect the GAPSS values, which reached 9.23±5.21 points in arterial thrombosis, 10.44±4.01 points in venous thrombosis, and 10.33±4.18 points in combined ones. Patients with recurrent thrombosis had higher GAPSS scores compared to patients without relapse: 8.19±5.25 points versus 11.00±3.65 points (p=0.01). There were no significant differences in GAPSS scores in obstetric pathology at different gestational ages.
GAPSS values ≥6 showed a higher risk of thrombosis recurrence: odds ratio 5.23 (95% CI 1.34–20.37). GAPSS scores ≥6 demonstrated the highest accuracy, with sensitivity and specificity of 72% and 66%, respectively. According to ROC analysis, the AUC value for GAPSS was 0.675 (95% CI 0.542–0.808; p=0.01).
Conclusion. The use of GAPSS makes it possible to identify patients at increased risk of recurrent thrombosis. GAPSS scores ≥6 have high sensitivity (72%) and specificity (66%), which can be used to stratify patients with PAPS into high and low risk groups for recurrent thrombosis.

 Objective: to evaluate the significance of clinical indicators, expression of pain syndrome genes cathepsin S and pro-inflammatory cytokines in peripheral blood for predicting the development of postoperative pain (POP)  in patients with hip osteoarthritis (HOA) before total arthroplasty (TA).
Material and methods. The study included 31 patients with stages III–IV of HOA (mean age 61.3±9.8 years) who underwent TA and 26 healthy volunteers (control group). Depending on the presence or absence of POP,  patients were divided into two subgroups: with POP (1st subgroup, n=12) and without POP (2nd subgroup, n=19). Patients were examined before and 6  months after surgery.
Prior to TA, the level of pain was determined in all patients using the visual analogue scale (VAS), as well as using the DN4 and PainDETECT neuropathic pain questionnaires. Functional status was assessed by the WOMAC index. The development of POP ≥30 mm according to VAS was assessed 6 months after TA.
Total RNA was isolated from whole blood and used to evaluate gene  expression for cathepsin S, interleukin 1 (IL1 ), tumor necrosis factor α (TNFα), and cyclooxygenase 2 (COX2) by quantitative real-time polymerase chain  reaction. 

Results and discussion. Complaints of pain persisted in 12 (38.7%) patients 6 months after TA. Before surgery, the expression of cathepsin S, IL1 , TNFα, and COX2 genes was significantly higher in patients of both subgroups compared to healthy controls. In addition, in patients with POP who were not satisfied with the outcome of TA, the rates of neuropathic pain according to  the DN4 questionnaire and cathepsin S gene expression were significantly  higher than in other patients. At the same time, there was no significant  difference in the expression of genes of pro-inflammatory cytokines in  patients of the studied subgroups before TA.
Conclusion. The development of POP in patients with HOA may be partially associated with its perception mechanisms disturbance, and an increase in the expression of the cathepsin S gene in the peripheral blood prior to TA can serve as its prognostic biomarker. 

Joint hypermobility (JH) is a common phenotype that can be both an independent clinical syndrome and a manifestation of connective tissue diseases. The pathogenesis of JH is not well understood. JH may be a predisposing factor in the development of musculoskeletal system pathology, so it is necessary to identify its molecular markers to prevent the formation of associated disorders.
Objective: to search for associations of five polymorphic variants of the ADAMTS5 gene with JH and connective tissue dysplasia (CTD).
Material and methods. A one-stage screening study of young people (n=181, mean age 21.86±0.22 years) was performed. We searched for associations of polymorphic variants of the rs226794, rs9978597, rs2830585, rs229077, rs229069 loci of the ADAMTS5 gene with JH, undifferentiated CTD, and their combinations. JH was determined by the Beighton scale, CTD – by a quantitative method. The study of polymorphic variants was carried out using real-time polymerase chain reaction. To compare qualitative features, Fisher's exact test with Yates’s correction for 2×2 contingency tables was used. The strength of associations was assessed using the odds ratio (OR), differences were considered significant at p<0.05, the correction for multiple comparisons was performed using the Benjamini–Hochberg method (false discovery rate, FDR).
Results and discussion. JH was detected in 128 (70.7%), signs of CTD – in 129 (71.3%) patients, including 115 (63.5%) patients in combination with JH. We found associations of the T allele and the TT genotype of the rs9978597 locus with the presence of JH (OR 5.00 and 7.81, respectively), CTD (OR 3.13 and 3.96), or their combinations (OR 6.33 and 10.23). An association of the GG genotype of the rs226794 locus with isolated JH was also found (OR 3.87).
Conclusion. The GG genotype of the rs226794 locus of the ADAMTS5 gene is a marker of isolated JH, the T allele of the rs9978597 locus is a marker of both isolated JH and CTD, and their combination.

Objective: to describe the rheumatic aspects of arthralgia associated with aromatase inhibitor therapy in a clinical case series.
Material and methods. The article presents a series of clinical cases – 16 patients (mean age 61±14 years) with histologically verified breast cancer (BC) and the onset of musculoskeletal pathology during hormone therapy with aromatase inhibitors (letrozole, anastrozole, exemestane). The laboratory and instrumental examination data, including ultrasound, joint X-rays, general and immunological blood tests with the determination of rheumatoid factor (RF), antibodies to cyclic citrullinated peptide (ACCP), CRP and antinuclear factor (ANF) are presented. The detected musculoskeletal pathology was compared with valid classification and/or diagnostic criteria for rheumatic diseases.
Results and discussion. In 10 (63%) cases, musculoskeletal pathology debuted in the first 3 months of therapy with aromatase inhibitors and in all patients it was represented by inflammatory manifestations, including clinically significant synovitis (n=13), tenosynovitis (n=12), enthesitis (n=5), morning stiffness in the joints >30 min (n=6) and inflammatory rhythm lower back pain (n=1). In 15 (94%) patients, ultrasound showed signs of synovitis (with increased vascularization in 5 cases), tenosynovitis in 15 (94%), and erosions in 1 (6%). X-rays showed osteoarthritis in 12 patients, and chronic erosive arthritis in 2. Increased ESR >30 mm/h and CRP level >5 mg/l were present in 6 (38%) and 7 (44%) patients, respectively; ANF (Hеp2) titer 1/160 was found in 8 (53%) out 15 examined patients. RF and ACCP were not detected in any case. During rheumatological examination, the following were diagnosed: polymyalgia rheumatica (n=1), undifferentiated arthritis (n=6), psoriatic arthritis (n=1), rheumatoid arthritis (n=1), recurrent proliferative synovitis of the knee joints with a pronounced exudative component (n=3), inflammatory lesions of periarticular tissues (n=4).
Conclusion. Based on the obtained data, it can be assumed that arthralgias induced by breast cancer hormone therapy may be a manifestation of inflammatory diseases of the musculoskeletal system. Given the heterogeneity of manifestations of musculoskeletal pathology in such patients, a complete rheumatological examination is necessary to establish the diagnosis and conduct adequate therapy.

Psoriatic arthritis (PsA) is a chronic progressive disease from the pondyloarthritis group of diseases. In recent years, there has been a significant increase in the incidence of PsA and cases of its severe course, with significant influence on the quality of life (QoL) of patients and early disability. Psoriatic Arthritis Quality of Life Questionnaire (PsAQoL) is the first quality of life questionnaire designed specifically for PsA that has not been previously validated in Russia.
Objective: to translate the original PsAQoL questionnaire into Russian and evaluate the psychometric properties of the Russian version.
Material and methods. The original PsAQoL was translated into Russian using two translational panels. In all, 12 local residents were included in the translation stage of the study. The external and logical validity of the questionnaire was assessed. Further, Russian-speaking patients with an established diagnosis of PsA (n=10) tested the questionnaire. A separate cohort of patients with PsA (n=50) was enrolled in a postal test retest study on 2 occasions, 2 weeks apart. Clinical data and the Medical Outcomes Study-Short Form (SF-36) questionnaire were used to assess convergent validity.
Results and discussion. The Russian version of PsAQoL was relevant, clear, and easy to complete (6.1 minutes on average). PsAQoL had high internal consistency (Cronbach's á=0.87) and excellent retest validity (r>0.85). PsAQoL scores correlated most strongly with the SF-36 General Health Scale (r=-0.68, p<0.01). PsAQoL score did not depend on sex and age (p>0.05). The PsAQoL could distinguish between groups of patients defined by self-reported general health status and self-reported severity of PsA.
Conclusion. The Russian version of PsAQoL proved to be understandable and easy to complete, as well as a reliable and valid tool for assessing the quality of life of patients with PsA.

Arthroscopic interventions are widely used to treat the consequences of the meniscus and anterior cruciate ligament (ACL) injuries. However, the long-term consequences of these surgeries are not always favorable and not in all cases allow to avoid the development of chronic pain and posttraumatic osteoarthritis.
Objective: to evaluate the incidence of persistent postoperative pain and the persistence of functional disorders in patients undergoing arthroscopic interventions on the menisci and ACL.
Material and methods. The study group consisted of 147 patients (60 women and 87 men, mean age 38.8±12.5 years) who underwent arthroscopic surgery on the knee joint (KJ) in the traumatology and orthopedic department of V.A. Nasonova Research Institute of Rheumatology in 2018– 2021. The condition of patients was assessed by telephone survey and/or online questionnaire. The pain and fatigue levels were assessed on numerical rating scale (NRS, 0–10), as well as the severity of functional disorders on the Lysholm scale (LS).
Results and discussion. Moderate or intense knee pain and increased fatigue (≥4 according to NRS) were noted in 11.3% and 14.7% of respondents, respectively. The state of the KJ according to LS in 35.3% of patients was assessed as excellent (95–100 points), in 29.3% – as good (84–94 points), in 21.3% – as satisfactory (65–83 points) and 14.0% – as unsatisfactory (≤64 points).
Conclusion. More than 10% of patients after arthroscopic operations on the knee joint experience moderate or severe pain and fatigue, satisfactory and unsatisfactory functional results are observed in 35.4% of cases.

Improving the treatment of knee (KN) osteoarthritis (OA) remains an urgent problem.
Objective: to compare the efficacy and tolerability of intra-articular (i/a) administration of long-acting glucocorticoids (GCs) and Armaviscon®Forte and Armaviscon®Platinum hyaluronates in patients with knee OA.
Material and methods. From November 2019 to April 2022, we examined and treated 60 patients (26 men and 34 women, mean age 41.32±3.71 years) suffering from primary KN OA. Study participants were randomly divided into three groups of 20 patients each. In the 1st group, a longacting GCs in combination with a local anesthetic was administered once i/a into the KN, in the 2nd – Armaviscon®Forte 2.3%, and in the 3rd – Armaviscon®Platinum 3%.
Patients were examined at baseline, on the 14th and 60th day after a single injection of the drug. The intensity of pain was assessed on a visual analog scale (VAS), the Lequesne index, the KOOS, WOMAC, and EQ-5D questionnaires were used. When studying tolerability, the number of adverse reactions (ARs) was taken into account.
Results. By the end of the observation period in the study groups, there was a decrease in the severity of pain and an increase in functionality with positive dynamics of the Lequesne and WOMAC indices. Both hyaluronic acid drugs had a positive effect on the functional state of the joints according to KOOS, improved the quality of life according to EQ-5D. The need for non-steroidal anti-inflammatory drugs in patients of the 2^nd and 3rd groups decreased down to their complete cancellation. No adverse events requiring discontinuation of therapy have been reported. The advantage of i/a administration of hyaluronates in comparison with long-acting GCs was shown.
Conclusion. In real clinical practice, in patients with symptomatic OA, i/a administration of hyaluronates allows obtaining more favorable results than the use of long-acting HA.

The article presents a review of the literature and a clinical observation of a patient with long-term anamnesis of primary Sjögren's syndrome (SS) in combination with sporadic inclusion body myositis (sIBM). The diagnosis of SS was confirmed in accordance with the Russian diagnostic criteria for SS 2001, as well as with the ACR 2012 and ACR/EULAR 2016 criteria. The diagnosis of sIBM was established on the basis of a characteristic clinical picture: the development of the disease in a woman after 50 years of age with slowly progressive asymmetric muscle weakness and a typical distribution, a moderate increase in the level of creatine phosphokinase (<10 norms for the entire observation period), the presence of a generalized primary muscle process according to needle electromyography, a typical picture of muscle involvement according to magnetic resonance imaging, and the ineffectiveness of high doses of glucocorticoids. The absence of histological confirmation does not contradict the diagnosis, since morphological examination of muscles in patients with a typical course of the disease fails to detect characteristic signs of sIBM in 20% of cases.

Currently, there is no effective pathogenetic therapy for sIBM. Understanding the mechanisms of sIBM development will allow to develop effective methods of its treatment.

Relapsing polychondritis (RPC) is a rare disease, its diagnosis presents certain difficulties. This is due to the absence of characteristic clinical manifestations at the initial stages of the disease, late diagnosis and difficulties in selecting adequate therapy.
The article presents a review of the literature on the diagnosis and treatment of RPC, as well as a clinical case with tracheobronchial tree and other organ systems involvement in the absence of classical auricular involvement.

In rheumatic diseases, the risk of infections development is higher than in the general population. The article describes a case of SARS-CoV-2 infection in a patient with rheumatoid arthritis (RA) and secondary AA-amyloidosis of the lungs, which led to a fatal outcome. In the context of the COVID-19 pandemic, RA patients with secondary AA-amyloidosis and pulmonary fibrosis are likely to represent a special risk group for developing a severe course of this infection with a fatal outcome.

Hyaluronic acid (HA) drugs are ingrained in complex treatment of osteoarthritis (OA). They have not only lubricant, but also anti-inflammatory properties, and ability to slow down the progression of OA. The article summarizes current data on the efficacy and safety of low molecular weight HA. The possibilities of using it both intra-articularly (i/a) and for the treatment of tendinopathies are considered. The authors present their own clinical experience in the treatment of patients with musculoskeletal (MS) pain syndromes.
It has been shown that i/a administration of HA can effectively reduce the intensity of pain and improve joint function, and when injected into the synovial sheaths of tendons, it can also relieve pain of extra-articular localization. The peculiarities of the surgical technique, preparing for manipulation and post-injection period management are described.
The existing evidence base indicates the expediency of active use of HA drugs not only for OA treatment, but also for various extra-articular musculoskeletal pain syndromes.

The review analyzes data on the course and outcomes of axial spondyloarthritis (axSpA) accumulated over the previous 2.5 years of the COVID-19 pandemic. The issues of clinical and immunological efficacy of vaccination against COVID-19 in this disease are considered. It was noted that the presence of axSpA, as well as treatment with tumor necrosis factor-á inhibitors and non-steroidal anti-inflammatory drugs, did not significantly increase the risk of COVID-19 infection and did not worsen its outcomes, apart from an increase in the incidence of venous thromboembolism. At the same time, it is assumed that anticytokine therapy for SpA may protect against severe COVID-19 course.
The data presented suggest that the benefits of vaccination in SpA far outweigh the potential harms associated with the development of adverse events. It has been shown that in patients with SpA, vaccination does not affect the activity of the inflammatory process, and biologic disease modifying antirheumatic drugs have almost no significant effect on the post-vaccination response.

Post-traumatic osteoarthritis (PTOA) is an inflammatory and degenerative disease that occurs as a result of the joint structures injury. It is a common pathology, accounting for approximately 12% of all cases of osteoarthritis (OA). PTOA often occurs in people of young productive age, progresses rapidly, causing chronic pain and increasing dysfunction. Individuals undergoing joint replacement for PTOA are, on average, 10 years younger than those with primary OA. The time interval from the moment of injury to the onset of typical PTOA radiological signs varies widely – from 1 year to 15–20 years.
The main injuries that cause PTOA are intra-articular fractures, anterior cruciate ligament injuries, meniscus rupture and dislocation of the patella of the knee joint, joint dislocations with damage to the ligamentous apparatus of the ankle and shoulder joints.
The pathogenesis of PTOA is determined by chronic inflammation accompanied by macrophage activation, hyperproduction of cytokines, primarily interleukin (IL) 1â, chemokines and growth factors, progressive destruction of joint tissue and degenerative changes (fibrosis, neoangiogenesis, osteophytosis).
Pathogenetic treatment of PTOA, which would stop the progression of the disease, has not been developed. The possibility of using inhibitors of IL1â, IL6, inhibitors of tumor necrosis factor á, glucocorticoids, hyaluronic acid, autologous cell based therapy is under study. The control of pain and inflammation in PTOA requires the prescription of traditional drugs that are widely used in the practice of managing patients with primary OA. In particular, the use of symptomatic delayed-acting agents, such as the injectable form of chondroitin sulfate, seems to be appropriate.