Vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) are a group of systemic autoimmune diseases characterized by necrotizing lesions of the walls of predominantly small vessels and the presence of ANCA against proteinase 3 or myeloperoxidase. However, an increase in ANCA levels can also be observed in other diseases, including autoimmune, malignant and infectious diseases, which complicates the interpretation of clinical and laboratory data and requires a differential diagnosis.

Objective: to evaluate the efficacy and safety of the type I interferon (IFN) receptor inhibitor anifrolumab (AFM, Safnelo^®) in patients with systemic lupus erythematosus (SLE) in real-life clinical practice over an observation period of 6 months.

Material and methods. The prospective 6-month study included 21 patients with SLE fulfilling the 2012 SLICC criteria, predominantly women (n=17,81%), median age – 31 [27; 46] years, disease duration – 9 [6.0; 11.0] years. Standard laboratory values and immunological markers of SLE were examined in all patients. The SLEDAI-2K index was used to determine the activity of SLE, and the severity of the mucocutaneous syndrome was assessed using the Cutaneous Lupus Disease Area and Severity Index (CLASI) index. Organ damage was assessed using the SLICC/ACR Damage Index (DI). After 6 months, the achievement of low activity was assessed according to the Lupus Low Disease Activity State (LLDAS) index

Results and discussion. At the time of inclusion in the study, the mean SLEDAI-2K activity index for the group was 8 [6.0; 10.0] points, the median CLASI index – 8.6±8.2 points, 81% of patients had skin and mucosal lesions, 66% had non-erosive polyarthritis, and high immunological activity was observed in all cases. Various irreversible organ damage was observed in 86 of patients. The average DI was 2.2±1.5 points. At the start of AFM therapy, all patients received glucocorticoids (GCs) at a mean dose of 10.7±5.6 mg/day, 52% of patients received a dose above 10 mg/day, 76% of patients continued to take hydroxychloroquine, and 33% of patients took immunosuppressants. Significant positive dynamics were observed with AFM therapy. The average CLASI index for the group after 3 months of treatment was 1.2±4.1 points, after 6 months – 0.3±1.2 points (p<0.0001). The SLEDAI-2K index fell significantly in the group on average from 8 [6.0; 10.0] to 2 [2.0; 4.0] points from the 3rd month of treatment (p<0.0001) and remained at the same level after 6 months. At month 6 of treatment, 13 (62%) of 21 patients met the LLDAS criteria for low disease activity. After the 3rd month of treatment, a significant decrease in antibodies against dsDNA was observed, which persisted for 6 months after the start of treatment. In the group as a whole, there were no significant changes in complement fractions values during the observation period. There was no increase in irreversible organ damage (DI – mean 2.2±1.5 points). The mean daily dose of GCs was significantly reduced from 10.7±5.6 mg/day to 7.5±4.0 mg/day (p<0.01) by the 3rd month and to 5.2±2.1 (p<0.001) by the 6th month of treatment. No infusion reactions were observed in any case. Adverse events occurred in 9 (42%) out of 21 patients, mainly herpes infections of varying severity, mainly after the 1st to 3rd infusions. In one case, severe herpes zoster was observed, so the drug was discontinued.

Conclusion. At a dose of 300 mg intravenously monthly AFM is a highly effective drug with a relatively good safety profile in patients with active SLE in whom autoantibodies are present and who do not respond adequately to standard therapy.

Objective: to present the experience of diagnosis, management and therapy with interleukin-1 inhibitors (iIL1) in patients with Chronic Infantile Onset Neurologic Cutaneous Articular/Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID) according to the Russian Federal Rheumatological Center data.

Material and methods. From 2007 to 2023, eight patients were included in the study (7 men) aged 10 months to 33 years, including 3 with a disease duration of more than 10 years (13, 17 and 33 years). Genetic testing was performed in all patients and mutations in the NLRP3 gene were identified in 6 cases.

Results and discussion. The age of onset of the disease ranged from 0 to 6 months. The delay in diagnosis and prescription of therapy ranged from 10 months to 33 years. All patients had the classic manifestations of CINCA/NOMID, including fever, rash, central nervous system (CNS) involvement, elevated ESR and CRP levels, 6 patients had articular manifestations, 7 had ocular manifestations and 6 had sensorineural hearing loss. Amyloidosis was detected in 1 case. All patients were prescribed iIL1. Anakinra was used in 6 patients (in 5 as the first line, in 1 as the second line therapy) with a positive response; subsequently 2 of these patients were switched to canakinumab once every 4 weeks (1 patient deteriorated and was readministered anakinra). Five patients received canakinumab (3 as first-line therapy, 2 as second-line therapy), 1 patient was switched to anakinra due to insufficient CNS response. The response to iIL1 therapy was positive in all patients, but incomplete in some of them due to the severity of the manifestations and the presence of irreversible organ damage.

Conclusion. Patients with CINCA/NOMID have a severe disease and a poor prognosis. In this context, early administration of iIL1 is necessary. In the case of CNS involvement, the use of anakinra is preferable, as it is characterized by better penetration of the blood-brain barrier and is therefore more effective. Later it is possible to switch the patient to canakinumab, however, to achieve a complete response, it is sometimes necessary to increase the dose of the drug and reduce the interval between doses.

Objective: to compare the clinical efficacy of seniprutug (BCD-180) and adalimumab (ADA) in the treatment of adults with active radiographic axial spondyloarthritis (r-axSpA).

Materials and methods. Based on the results of a previously conducted systematic review, an unanchored matching-adjusted indirect comparison (MAIC) was performed, adjusting for confounding factors. The analysis was based on the results of randomized placebo-controlled clinical trials of seniprutug (BCD-180-2/ELEFTA, NCT05445076) and ADA (ATLAS, NCT00085644) that met the selection criteria. We chose ASAS40 and ASAS20 measurements at week 24 as efficacy outcomes. Initial BASDAI and BASFI indices, proportion of women in the study population, time from disease onset, and baseline C-reactive protein (CRP) levels were considered as confounders.

Results and discussion. The MAIC showed a statistically significant advantage in the clinical efficacy of seniprutug (BCD-180) over ADA. When adjusted, the odds ratios (OR) with 95% confidence intervals (CI) for seniprutug (BCD-180)/ADA were 1.86 (1.15; 3.02) and 2.21 (1.34; 3.72) for ASAS40 and ASAS20, respectively, at week 24.

Conclusion. The MAIC demonstrated statistically significant superiority of seniprutug (BCD-180) over ADA on the key efficacy endpoints ASAS40 and ASAS20 at week 24 in adults with active r-axSpA. The inclusion of the innovative domestic drug seniprutug into treatment paradigm of active r-axSpA will potentially reduce the socio-economic burden of this disease by providing an affordable, effective and safe therapy while optimizing healthcare costs

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.

Objective: to compare the course of “rheumatoid disease” in multimorbid patients with and without interstitial lung disease (ILD).

Material and methods. Two groups were formed of 1034 patients with active rheumatoid arthritis (RA) who met the 2010 ACR/EULAR criteria: one group with ILD identified by high-resolution computed tomography of the lungs (n=82) and another – without ILD (n=900). In all patients, estimated glomerular filtration rate (eGFR) was determined using the Cockroft–Gault formula. The presence and stage of chronic kidney disease (CKD) was assessed depending on the eGFR level: stage I CKD was diagnosed in eGFR <89 ml/min, stage II (subclinical) – 60<eGFR <89 ml/min, stage IIIa (clinical) – 45<eGFR<59 ml/min and IIIb – 30<eGFR<45 ml/min.

Results and discussion. Arterial hypertension (p=0.004), cerebrovascular disease (p=0.0001), diabetes mellitus (p=0.04), obesity, psoriasis (p=0.009) and stage II–III CKD (p=0.04) were more frequently observed in the group with ILD.

Conclusion. The combination of CKD and ILD frequently found in patients with RA and the known similarity and close interrelationship of the pathogenesis of these diseases, suggest a type of “renal-pulmonary syndrome” associated with a specific variant of RA.

The vitamin B₁, B₆ and B₁₂ complex (VBC) is frequently used to treat acute and chronic low back pain.

Objective: to investigate the effect of a combination of a non-steroidal anti-inflammatory drug (NSAID) and a VBC on the main manifestations of nociceptive system dysfunction in patients with combination of osteoarthritis (OA) and chronic non-specific low back pain (NSLBP).

Material and methods. The study group consisted of 99 patients (82% women, mean age 63.6±17.2 years) with OA of various localization and

NSLBP who had moderate to severe pain (≥4 on a numerical rating scale, NRS 0–10). All patients received etoricoxib 60 mg/day (up to 14 days) and a course of intramuscular (IM) injections of VBC (a drug for parenteral administration containing solutions of thiamine 100 mg, pyridoxine 100 mg, cyanocobalamin 1.0 mg and lidocaine 20 mg) 2.0 ml №10. Treatment outcome was assessed after 14 days.

Results and discussion. During treatment, the vast majority of patients showed a significant improvement: the median severity of pain on movement (NRS) decreased from 6.3 [5.0; 8.0] to 3.7 [3.0; 5.0], p=0.0001; functional impairment  –  from 3.8 [2.0; 6.0] to 2.2 [1.0; 3.0], p=0.001; fatigue – from 5.6 [4.0; 8.0] to 3.5 [0.0; 2.0], p=0.0001. 71.6% of patients rated the treatment results as good or excellent. Six patients had adverse reactions: 2 – local pain at the site of the intramuscular injections, 1 – arterial hypertension, 3 – epigastric pain. No serious adverse events were recorded.

Conclusion. The combined use of NSAIDs and VBC can provide significant improvement in patients with a combination of OA and NSLBP.

The most important factors that determine the risk of fractures are not only the presence of osteoporosis (OP), but also the risk of falls. In addition to sufficient physical activity, medications are needed that have a positive effect on the strength and functional performance of the skeletal muscles. Vitamin D supplements play a key role in this therapeutic approach.

Objective: to investigate the tolerability, safety and efficacy of the generic drug Alfacalcidol Canon on the functional status of patients with reduced bone mineral density in clinical practice.

Material and methods. The study involved 30 women with OP or osteopenia who were at high risk of falls (mean age 68.0±7.2 years) and who were administered Alfacalcidol Canon at a dose of 1 mcg/day for 3 months. The follow-up period included 4 visits to the clinic, during which laboratory tests were performed and falls and fractures, adverse reactions and patients' adherence to treatment were registered. At the first and last visits, quality of life was assessed using the EQ-5D and the functional status of the skeletal muscles was examined. At the 4th visit, the tolerability of the medication was assessed.

Results and discussion. After 3 months of therapy, an increase in muscle strength of the dominant arm was observed by an average of 15.4% (p=0.00002). The time for the "Chair Stand Test" and the "Time up and go" test decreased by 17.4% (p=0.000012) and 13.6% (p=0.00004) respectively. The gait speed test increased on average by 13.9% (p=0.000013), it increased in 26 (86.7%) women. The SPPB test score increased on average by 12.5% (p=0.000001), and achievement of the best indicators was found in 23 (76.7%) women. There were no cases of hypercalcemia, hyperphosphatemia and hypermagnesemia, but 1 case of hypercalciuria was detected. Falls occurred in 2 women (6.7%) who had fallen in the previous year, one of whom suffered a fracture of humeral neck. Adverse reactions were observed in 3 (10 %) women, one of whom stopped taking the drug. 90% of the patients tolerated the treatment excellently. The average compliance rate was 94.3%.

Conclusion. Alfacalcidol Canon is a safe and effective drug that can be used to improve the physical performance of skeletal muscles in patients with a high risk of falls.

Libman–Sachs endocarditis (nonbacterial thrombotic endocarditis, NBTE) may be one of the cardiac manifestations of systemic lupus erythematosus (SLE). It is characterized by the presence of sterile platelet thrombi on previously normal heart valves. The diagnosis of NBTE is difficult as it is often asymptomatic until complications such as systemic emboli or valvular dysfunction occur. Patients with Libman–Sachs endocarditis and SLE are treated with immunosuppressive and anticoagulant therapy and, if it is ineffective, surgical treatment.

A clinical case of SLE and Libman–Sachs endocarditis is presented. The clinical manifestations, diagnostic methods and treatment of NBTE in SLE are discussed. The difficulties in differential diagnosis and selection of optimal treatment tactics faced by physicians are highlighted.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology characterized by the overproduction of autoantibodies against various components of the nucleus of the patient's own cells with the development of immunoinflammatory tissue damage. In recent years, more and more data have accumulated on the involvement of neutrophils in the development of the clinical symptoms of SLE, and DNA-containing structures and neutrophil extracellular traps (NETs) playing an important role in this process. Effective neutralization of NETs in SLE can be achieved by removing circulating proteins and molecules associated with NETs from the bloodstream by selective plasma sorption of DNA using the NucleoCapture Device.

This article describes the case of a patient who underwent three plasma sorption sessions aiming to suppress the activity of SLE. During the therapy, significant positive dynamics were achieved: the SLEDAI-2K index decreased from 32 to 12 points, the number of leukocytes in the blood normalized, renal function improved, and the immunological activity of the disease decreased.

Drug-induced hypersensitivity syndrome (DIHS) or drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially fatal systemic reaction characterized by multiorgan damage involving the liver, hematopoietic system and skin, and heterogeneous manifestations of fever, rash, lymphadenopathy and eosinophilia with unpredictable course.

We describe a 41-year-old female patient who developed DRESS syndrome after taking sulfasalazine prescribed for non-radiographic axial spondyloarthritis. Treatment with intravenous and then oral glucocorticoids was effective. A review of the literature on this topic is presented.

Lupus nephritis (LN) occurs in 35–60% of patients with systemic lupus erythematosus (SLE), often in the early stages of the disease. LN is one of the most severe manifestations of SLE and, if not treated promptly and effectively, can lead to rapid and severe loss of kidney function. Despite modern pharmacotherapy, 5–20% of patients develop end-stage renal failure within 10 years of diagnosis of LN. The main principle of LN therapy is to prevent deterioration of renal function, but a consensus on outcome assessment criteria and clinically relevant short- and long-term goals for LN therapy has not yet been reached. There is increasing evidence of the importance of repeat kidney biopsies to assess the outcomes of the initial phase of therapy and to determine the long-term prognosis of renal failure. It is believed that the information obtained from repeat biopsies can help to make optimal treatment decisions and thus increase the likelihood of achieving a complete renal response in the short term and a more favorable renal prognosis in the long term.

We describe a clinical case of a young patient with highly active SLE and morphologically confirmed class IV-S LN. Repeat biopsy and clinical and laboratory examination, had confirmed a clinical and laboratory remission of the disease on the background of the therapy. This made it possible to reduce the dose of methylpednisolone and cytostatic drugs (mycophenolate mofetil) and to discontinue therapy with biologic diseasemodifying antirheumatic drug (rituximab).

Patients with juvenile-onset systemic lupus erythematosus (jSLE) account for up to 25% of all SLE patients. The main difference between jSLE and SLE in adults is the greater role of genetic factors in the pathogenesis, higher activity, earlier development of complications and the need for more aggressive immunosuppressive therapy, which allows us to consider the onset of the disease in childhood as a special phenotype of SLE. The relevance of the study of jSLE arises from the variability of clinical manifestations and the unpredictability of the course, the difficulty of early diagnosis, the rapid development of organ damage and the unfavorable life prognosis.

The article presents the most important modern data on the diagnosis, classification, features of the clinical picture and treatment approaches of jSLE from a practical point of view.

Osteoporosis (OP) is a common disease leading to low-trauma fractures and is a serious medical and social problem. Often a fracture is the first clinical manifestation of OP that has been asymptomatic for a long time, necessitating the development of methods for early detection and risk assessment of this disease. OP is a multifactorial disease with a strong hereditary component. However, as the data from the study of genetic factors show, only 15% of the heritability of this trait can be explained. In this context, the focus of research is shifting to the area of epigenetic regulation, which controls gene activity without altering the primary structure of the DNA. One of the most promising mechanisms of epigenetic control is methylation, which affects DNA as well as RNA and histones. The characteristics of these mechanisms and the possibilities of their use for the diagnosis and treatment of OP are presented in this review.