Systemic sclerosis (SSc) is characterized by multisystem involvement and represents a serious clinical challenge for physicians and patients. Among the visceral manifestations of SSc, primary cardiac involvement is one of the most severe and is often diagnosed late. Biomarker assessment can make a major contribution to the early diagnosis of cardiopathy in SSc.

The second part of the article is devoted to the potential use of various cytokines and other molecules for diagnosis and prognostic assessment of primary cardiac involvement associated with SSc. 

Objective: to develop a population-based mortality model for patients with systemic sclerosis (SSc) and to assess the disease burden (mortality indicators) for the Russian Federation.

Material and methods. A systematic review was conducted in the PubMed and Embase databases. Eleven real-world clinical practice studies were selected. Data analysis was performed in R v.4.3.0 using a random-effects meta-analysis model and meta-regression analysis. Covariates included the proportion of patients with diffuse disease and the proportion of patients with interstitial lung disease. For forecasting indicators for the Russian Federation, the proportions of patients were assumed to be 40% and 60%, respectively. Risk of bias was assessed using the ROBINSIv2 tool.

Results and discussion. Based on bivariate meta-regression analysis, SSc mortality in Russia was estimated at 26.1 deaths per 1000 patientyears (95% confidence interval, CI 17.3–39.3). The standardized mortality ratio was 4.2 (95% CI 2.6–6.5), indicating a 4.2-fold higher risk of death compared with the general population. The obtained estimates are consistent with international data and confirm the determining role of diffuse disease and lung involvement in shaping mortality.

Conclusion. SSc in Russia is associated with a high risk of premature mortality. The developed meta-regression model provides valid preliminary estimates of disease burden in the absence of a national registry. The results emphasize the need for early detection of lung involvement, standardized screening, and establishment of a national registry to monitor disease course and treatment effectiveness. 

Objective: to study the association of serum calprotectin (CLP) with clinical and laboratory manifestations of systemic lupus erythematosus (SLE).

Material and methods. The study included 53 patients with a definite diagnosis of SLE and a median disease duration of 6 [1; 12] years; SLEDAI-2K=10 [6; 18] points. The control group consisted of 40 apparently healthy individuals matched for sex and age. Serum CLP concentration was measured by enzyme-linked immunosorbent assay using a Bühlmann Laboratories AG (Switzerland) reagent kit according to the manufacturer’s instructions.

Results and discussion. In patients with SLE, serum CLP levels were significantly higher than in the control group (3.7 [2.1; 7.4] vs 2.36 [1.77; 3.51] μg/ml; p=0.004). Patients with moderate and high SLE activity had higher CLP concentrations than those with low activity (5.61 [2.32; 8.28] vs 2.36 [1.56; 3.72] μg/ml, respectively; p=0.034). Higher CLP concentrations were associated with the presence of arthritis and elevated CRP levels, whereas lower concentrations were associated with leukopenia and neutropenia.

Conclusion. In SLE, a significant increase in serum CLP is observed, which is closely associated with SLE activity, the presence of arthritis, and elevated CRP. This supports an important role of the neutrophil-mediated immunity and NETosis in SLE pathogenesis. 

Lupus nephritis (LN) largely determines the severe course and unfavorable life prognosis of patients with systemic lupus erythematosus (SLE) and remains one of the important therapeutic problems. LN is particularly severe in African Americans, Hispanics, and Asians, which is attributed to genetic characteristics of these ethnic groups. 

Objective: to study clinical and laboratory manifestations, disease course variants, and prognostic factors for LN development in patients from Kyrgyzstan.

Material and methods. The study included 800 patients with a definite diagnosis of SLE meeting the SLICC classification criteria. LN was diagnosed according to the ACR criteria (2004). The KDIGO criteria (2012) were used to define acute kidney injury (AKI). The KDIGO (2012) chronic kidney disease (CKD) classification was applied to determine the degree of decrease in glomerular filtration rate and the severity of proteinuria.

Results and discussion. Kidney involvement was diagnosed in 295 (36.9%) of 800 SLE patients. Patients with LN had a high frequency of mucocutaneous syndrome (91.2%), serositis (88.5%), alopecia (66.4%), central nervous system (CNS) involvement (42.7%), lymphopenia (35.2%), and hypocomplementemia for C3 (55.6%) and C4 (53.2%). In 45.8% of cases, severe CKD stages (G3a, G3b, G4, and G5) were observed, with nephrotic syndrome developing in 22.7% and AKI in 7.8% of patients.

Conclusion. The frequency of LN in SLE in the Kyrgyz cohort was 36.9%, and women predominated among patients with LN (89.5%). In 45.8% of cases, severe forms of LN were observed with the development of AKI (in 7.8% of patients) and end-stage renal disease (in 4.7%). Factors associated with an increased risk of LN in the Kyrgyz cohort include acute course of SLE, high disease activity, fever, mucosal involvement, CNS involvement, lung involvement, serositis, pulmonary arterial hypertension, leukopenia/lymphopenia, positivity for anti-Sm antibodies, and hypocomplementemia for C3 and C4. 

Ocular involvement is a common and potentially disabling manifestation of antineutrophil cytoplasmic antibody-associated systemic vasculitis (AAV). Available data on the frequency and pattern of ocular involvement are focused mainly on granulomatosis with polyangiitis (GPA), whereas they remain insufficiently studied in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA).

Objective: to analyze the frequency and patterns of ocular involvement in GPA compared with other AAV.

Material and methods. Data from 174 patients with AAV were analyzed, including 113 with GPA, 35 with MPA, and 26 with EGPA, who were followed up at the V.A. Nasonova Research Institute of Rheumatology from 2023 to 2025. Demographic and clinical parameters were analyzed.

Results and discussion. Ocular involvement was identified in 55 (31.6%) of 174 patients, most often in GPA (n=50, 44.2%), less often in MPA (n=4, 11.4%) and EGPA (n=1, 3.8%). The most common manifestations were episcleritis/scleritis (n=36, 65.5%), peripheral corneal ulceration (n=11, 20%), uveitis (n=16, 29%), and orbital pseudotumor (n=13, 23.6%). In 31 (56.4%) patients, one type of ocular involvement was present; in 24 (43.6%), two or more. Ocular involvement correlated positively with anti-proteinase 3 (PR3)-ANCA levels and negatively with anti-myeloperoxidase (MPO)-ANCA concentrations. The most common ocular complication was cataract (n=23, 42%). Irreversible vision loss was observed only in patients with GPA (n=5, 4.4%).

Conclusion. Ocular involvement was detected in one third of patients with AAV, especially in GPA with PR3-ANCA-positive status. The frequency of irreversible vision loss remains high.

 Objective: to evaluate the efficacy of the nutraceutical Teraflex Ultra, a combination of glucosamine (G, 1500 mg), chondroitin sulfate (CS, 1000 mg), undenatured type 2 collagen (UC-2, 40 mg), a B-vitamin complex, vitamin C (100 mg), and ginger root extract (300 mg), in patients with the metabolic phenotype of knee osteoarthritis (OA).

Material and methods. This prospective randomized study included 29 women aged 40–75 years with a definite diagnosis of knee OA stage I–III according to Kellgren–Lawrence (established in accordance with the ACR classification criteria) and metabolic syndrome, pain on walking ≥40 mm on the visual analog scale (VAS), who provided informed consent. Mean age was 62.4±6.9 years. Median baseline VAS pain was 63 [54; 75] mm. All patients received the nutraceutical Teraflex Ultra orally, 2 capsules twice daily for 6 months; the subsequent 3 months constituted the follow-up period with assessment of the carryover effect. Study duration was 9 months.

Results and discussion. The study results demonstrated a statistically significant clinical effect of the nutraceutical in patients with metabolic knee OA during the 6-month treatment course and the subsequent follow-up period. After 1 month of treatment, a significant (p<0.01) reduction in pain on walking in the analyzed knee according to VAS was observed; it persisted throughout the treatment period and 3 months after completion. A reduction of pain to <40 mm on VAS after 1 month of therapy was achieved in 48.3% of patients, after 3 months in 66.7%, and after 6 months in 81.5%. During follow-up, in most patients (77.7%) VAS pain remained <40 mm. Treatment was associated with significant improvement in all WOMAC parameters: decreased pain (baseline 246 [159; 320] mm; 1 month 173.5 [116; 199] mm; 3 months 119 [70; 186] mm; 6 months 84 [48; 157] mm; 9 months 73 [42; 106] mm; p<0.01 for all comparisons vs baseline); decreased stiffness (baseline 101 [63; 129] mm; 1 month 69.5 [36.5; 90] mm; 3 months 46 [23; 85] mm; 6 months 36 [19; 74] mm; 9 months 24 [15; 51] mm; p<0.01 for all comparisons vs baseline); decreased functional limitation (baseline 932 [610; 1085] mm; 1 month 660.5 [459; 767] mm; 3 months 463 [323; 748] mm; 6 months 309 [211; 559] mm; 9 months 265 [189; 440] mm; p<0.01 for all comparisons vs baseline). In all patients during the 6 months of treatment and the followup period, significant improvement in KOOS scores (p<0.01), quality of life according to EQ-5D, and patient global health assessment by VAS (p<0.01) was observed. As a result of therapy, most patients discontinued regular use of nonsteroidal anti-inflammatory drugs (NSAIDs).

Conclusion. In patients with the metabolic phenotype of OA, the nutraceutical Teraflex Ultra (a combination of CS, G, UC-2, B vitamins, vitamin C, and ginger root extract) demonstrated a significant clinical effect accompanied by reduced pain, improved joint function and quality of life, and decreased need for NSAIDs. 

Objective: to identify possible predictors of achieving remission in patients with rheumatoid arthritis (RA) during levilimab (LVL) therapy in realworld clinical practice.

Material and methods. In a retrospective observational study conducted at the G.G. Kuvatov Republican Clinical Hospital (Ufa) from January 2023 to January 2025, the efficacy of LVL therapy was assessed over time using clinical and laboratory parameters at weeks 12, 24, 52, and 104 after treatment initiation. The analysis included 37 patients with RA (30 women and 7 men; mean age 43±12.6 years).

Results and discussion. Mean DAS28-CRP and DAS28-ESR values at LVL initiation were 6.6±0.8 and 6.9±0.8, respectively. By the end of follow-up, all 37 patients achieved remission according to at least one criterion: DAS28-CRP, DAS28-ESR, CDAI, or SDAI. Multiple linear regression analysis identified factors influencing RA activity according to DAS28-CRP at week 104. Age (p=0.005), disease duration since RA diagnosis (p=0.014), fatigue severity (p=0.022), and patient global assessment of disease activity on the visual analog scale (VAS; p=0.017) had a statistically significant positive effect on the final DAS28-CRP value. The greatest negative impact was exerted by pain intensity on VAS (p=0.001). A favorable safety profile of LVL was noted.

Conclusion. Age, disease duration since RA diagnosis, fatigue severity, patient global assessment of disease activity, and pain intensity on VAS are the most significant prognostic criteria for achieving remission in patients with RA receiving LVL. 

Antineutrophil cytoplasmic antibody-associated systemic vasculitides (AAV) are a heterogeneous group of multisystem diseases characterized by inflammation and necrosis of the vessel wall of small- and medium-sized vessels. The international community subdivides them into three nosological forms: granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. The epidemiology of this disease in children has been insufficiently studied. In the Russian Federation, there is no unified registry of patients with this diagnosis; in foreign studies, only a very limited view of the problem is presented due to small sample sizes and difficulties in defining inclusion criteria for the study group. Early diagnosis remains a challenging clinical task.

The article describes two clinical cases of GPA in children and presents methodological approaches to disease diagnosis and treatment. A comparative analysis of GPA development in 17- and 10-year-old female patients was performed. Diagnostic methods for GPA in the absence of ANCA and the need to implement lung biopsy as the “gold standard” in ANCA-negative status are discussed. 

The pathogenesis of autoimmune rheumatic diseases is a complex multifactorial process based on activation of various components of the immune system. One of the approaches to the treatment of these diseases is extracorporeal therapy technologies, which are techniques aimed at removing from the blood the components involved in pathogenesis and leading to tissue damage. This review discusses various therapeutic apheresis techniques used for the treatment of systemic lupus erythematosus. 

Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by generalized microangiopathy and fibrosis of the skin and internal organs. The gastrointestinal (GI) tract is the most frequently affected organ system in SSc, and signs of GI involvement often occur at early stages of the disease. Currently, approved treatment options for GI involvement in SSc are limited, while approaches to treatment of different GI segments’ differ fundamentally. Despite its high prevalence, gastroenterological complications of SSc are insufficiently covered in the literature. This review analyzes current methods for the diagnosis and treatment of GI involvement in SSc. 

Takayasu arteritis (TA) is a rare inflammatory disease that primarily affects large and medium-sized arteries. The clinical picture is characterized by a wide spectrum of manifestations; in the early stages the disease is usually asymptomatic, and later it may progress to ischemic complications. Ischemic symptoms may vary depending on the affected vessel. Neurological manifestations of TA are diverse. The most dangerous of these is stroke, which often represents the main cause of disability and death in these patients. Mechanisms of stroke in TA include steno-occlusive lesions of intracranial or extracranial vessels due to arterial thrombosis and vasculitis, aneurysm, dissection of the carotid or vertebral artery, embolism, etc. In rare cases, TA may be complicated by hemorrhagic stroke due to aneurysmal dilatation of cerebral vessels and/or arterial hypertension. Early identification of TA in young patients with acute cerebrovascular events is important in clinical practice. 

Patients with psoriasis (PsO) and psoriatic arthritis (PsA) are characterized by a high prevalence of concomitant obesity and metabolic disorders, and an increased body mass index (BMI) increases the likelihood of PsA development in patients with PsO. Obesity, as well as PsO/PsA, is associated with increased cardiovascular risk and reduced quality of life. Achieving minimal disease activity in PsO/PsA in obese patients may be challenging. Effective and sustained BMI reduction (regardless of the method) increases the likelihood of achieving remission and enhances the efficacy of tumor necrosis factor α (TNFα) inhibitors.

This review presents the results of studies on the effects of the most commonly used drug class for obesity treatment — glucagon-like peptide 1 receptor agonists — on PsO/PsA activity, as well as their combined use with biologic agents. 

Paradoxical psoriasis (pPsO) is an exacerbation or de novo development of psoriasis (PsO) during treatment with drugs that are expected to control it. It is observed in approximately 5% of patients receiving tumor necrosis factor α inhibitors and less often in those receiving interleukin-17 inhibitors (IL-17i). To date, several dozen cases of pPsO have been described during IL-17i therapy in patients with PsO and only isolated cases in patients with ankylosing spondylitis (AS).

We observed 2 patients (a man and a woman) with AS who developed three episodes of pPsO during therapy with three different IL-17i: secukinumab (SEC), netakimab (NTK), and ixekizumab (IXE). In one case, typical plaque PsO developed during SEC therapy; in another, palmoplantar pustulosis appeared 1 month after the first NTK administration and regressed after drug discontinuation. A recurrent exacerbation was observed 2 years later during IXE therapy. A pathological description of a skin biopsy specimen is presented.

The article discusses the frequency, probable causes of development, and management approaches for pPsO. 

Febuxostat was originally developed as a treatment for hyperuricemia in patients with gout; however, studies in recent years have demonstrated broader therapeutic potential beyond its urate-lowering effect. The main mechanism of action of febuxostat is inhibition of xanthine oxidase activity, which leads to a reduction in serum uric acid levels. It is assumed that other mechanisms of action of not lesser importance are related to involvement in immune processes and contribute to suppression of both local and systemic inflammation; it also has an antioxidant effect. The drug should be considered safe in patients with cardiovascular diseases and is characterized by low hepatotoxicity. In addition to the established renoprotective effect of febuxostat, consisting in slowing the progression of chronic kidney disease, its potential use in other diseases is currently being considered, in particular in osteoarthritis, nervous system and pulmonary diseases, inflammatory bowel diseases, and COVID-19. 

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with marked clinical and immunological heterogeneity, which complicates objective assessment of its activity. The objective of this work was to adapt and translate into Russian the SLE activity index, the Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS). Forward and back translation were performed, the text was reviewed by experts, and a test version was piloted. The final version accounts for clinical and laboratory manifestations of SLE and is convenient for use in routine practice. The Russian-language version of SLE-DAS will help improve objective assessment of disease activity and enhance the quality of patient management. 

The results of the Russian interdisciplinary consensus on the definitions of “difficult-to-manage” and “difficult-to-treat” psoriatic arthritis/psoriasis are presented. Rheumatology and dermatovenereology experts participated in a Delphi-method voting process.