Classification Criteria Research Articles

The effect of rituximab therapy and the risk of hypogammaglobulinamea on immunoglobulin levels in patients with rheumatoid arthritis

Background. Rituximab (RTX) is a biological monoclonal antibody targeting the CD20 antigen on B cells, effectively decreasing disease activity and progression in patients with rheumatoid arthritis (RA). RTX treatment can lead to a decline in plasma cells that produce immunoglobulin, potentially resulting in hypogammaglobulinemia (HGG). This study aims to assess the effect of RTX on immunoglobulin levels and determine the prevalence of HGG in RA patients after receiving different doses and cycles of RTX. Additionally, immunoglobulin levels were compared between patients treated with RTX and those treated with traditional disease-modifying antirheumatic drugs (DMARDs). We also sought to identify patient, disease, and therapy factors associated with HGG to evaluate the importance of monitoring immunoglobulin levels. Method. A single-center observational cross-sectional study was conducted at Baghdad Teaching Hospital between November 2023 and May 2024. Patients with RA were recruited and divided into two groups. The first group consisted of 45 RA patients receiving RTX, and the second group included 45 patients receiving DMARDs. All patients met the American College of Rheumatology (ACR) 1987 or ACR/European League Against Rheumatism (EULAR) 2010 classification criteria for RA. Patients were excluded if they had received RTX for conditions other than RA or if they had been treated with RTX for less than six months. Quantitative IgG and IgA levels were measured via enzyme-linked immunosorbent assay (ELISA) to evaluate the effect of RTX on immunoglobulin levels and compare it with the effect of DMARDs. HGG was defined as an immunoglobulin level below two standard deviations from the mean of age-matched healthy controls. Results. Ninety patients were enrolled in the study, 45 of whom were treated with RTX. The mean number of RTX cycles was 3.67, ranging from 2 to 10 cycles. A significant decrease in immunoglobulin levels was observed post-RTX therapy, with 84.4% of patients exhibiting low IgG levels (<5 μg/ml), leading to HGG. The IgG levels in RTX-treated patients ranged from 4.3 to 22.0 μg/ml, with a mean of 7.28 ± 3.95 μg/ml. In contrast, patients treated with DMARDs maintained normal immunoglobulin levels similar to healthy individuals, with IgG levels ranging from 19.7 to 43.7 μg/ml. RTX also affected IgA levels, but to a lesser extent than IgG. Conclusion. Lower immunoglobulin levels following RTX therapy frequently result in HGG, emphasizing the importance of immunologic surveillance before and after treatment. DMARD therapy did not affect immunoglobulin levels, and concurrent use of DMARDs with RTX offered no protection against HGG. Additionally, HGG was not associated with an increased incidence of infections.

Are ultrasound salivary parenchymal lesions more severe in primary Sjögren patients with a longer disease duration? A cross-sectional study.

Salivary gland ultrasound (SGUS) has an interest in primary Sjögren's disease (pSD) for diagnosis, but the evolution of parenchymal lesions over time is unknown. The objective of this study was to assess the severity of ultrasound abnormalities in relation to pSD duration from the time of buccal dryness onset. In this cross-sectional international multicentre study, patients with pSD according to the 2002 or 2016 ACR/EULAR classification criteria were included. Parenchymal abnormalities were classified according to the semiquantitative score as defined by OMERACT. Patients were separated into 4 groups (Group A: < 5 years, Group B: 5-9 years, Group C: 10-20 years, and Group D: > 20 years from the onset of buccal dryness). The association between disease duration groups and SGUS lesions was quantified in terms of odds ratios and 95% confidence intervals. A total of 247 patients were consecutively included between May 2019 and February 2022. Eighty-nine percent of patients had a focus score ≥1/4 mm2, and 85% had positive anti-Ro/SSA. pSD duration was associated with a pathological OMERACT score (score 2 or 3): OR for 5-year duration: 1.23 [95% CI 1.04; 1.47], p= 0.0383). Considering each US item, the only statistical association with pSD duration was found regarding the presence of hyperechoic bands (25% or more): OR for five-year duration 1.18 [95% CI 1.03; 1.36], p= 0.038), independent of an older age. pSD duration was associated with the presence of hyperechoic bands, but not with hypoechoic areas, suggesting a progressive fibro-adipose evolution.

2019 EULAR/ACR classification criteria for SLE score predicts future lupus hospital admission and costs.

To test the ability of the 2019 EULAR/ACR Classification Criteria for SLE score to predict lupus related hospitalization and overall cost of hospitalization. 217 University of Kentucky patient records that met our preliminary inclusion criteria, 44 patients were selected by a random number generator algorithm for a thorough chart review to collect data needed for calculation of the 2019 EULAR/ACR Classification Criteria for SLE score. Total hospitalization cost was calculated by using hospital adjusted expenses per inpatient day data, which estimates the expense incurred by the hospital to provide services and thus removes the variability of charges and reimbursements introduced by insurance type. Patients with a score of 19 or more had increased risk of hospitalization in at least the 6months after initial outpatient visit as compared to their counterparts with scores less than 19 [p= .069]. The odds of being hospitalized for lupus among those with initial score ≥19 was 5.71times higher than for those with score <19. Patients who scored 19 or less at initial visit had a mean hospitalization cost of $14,499, whereas those scored >19 had mean hospitalization cost of $28,725. This study adds to the growing evidence that 2019 EULAR/ACR Classification Criteria score for SLE can be used as a surrogate marker to assess disease severity. The weighted 2019 EULAR/ACR Classification Criteria for SLE score offers a promising tool beyond its primary objective to find true lupus cases for research and clinical trials.

Biological hallmarks of systemic sclerosis are present in the skin and serum of patients with Very Early Diagnosis of SSc (VEDOSS).

The Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR study showed that, despite not showing any clinical sign of disease, patients with Raynaud's and antinuclear antibodies and/or capillaroscopy abnormalities often progress to systemic sclerosis (SSc) within 5 years. We aimed to determine whether VEDOSS biosamples show biological SSc activity pre-clinically. Skin biopsies were histologically analysed. Dermal fibroblasts analysed by RT-qPCR and gel contraction assays. Sera were assayed by Luminex (CXCL10) or ELISA (ELF score). Healthy controls (HC) and SSc biosamples were used for controls. Overall, 114 consecutive VEDOSS patients were enrolled, of which 36 consented to have skin biopsies. Skin biopsies showed a variable but overall increased collagen staining and skin thickness, increased perivascular infiltrate of CD45 positive cells and CXCL10 expression. In vitro, VEDOSS dermal fibroblasts showed increased profibrotic gene expression and contractibility compared with HC. Increased serological CXCL10 (mean [SD]; 75.90 [107.80] vs HC 39.90 [26.27] pg/ml, p = 0.02) and ELF score was evident in VEDOSS compared with HC (8.19 [0.78] vs 8.55 [0.79], p = 0.04). In longitudinal analysis of a median of 27.5 (IQR 44.5) months, 14.9% of VEDOSS patients progressed to SSc. Baseline CXCL10 serum concentration was significantly higher in the VEDOSS patients that progressed (2-fold increase, p = 0.0071) and correlated with ELF score (R = 0.3096, p = 0.0065). Despite not fulfilling classification criteria, VEDOSS patients show SSc-linked fibrosis and immunity dysregulation both within the tissue and sera, supporting a biological diagnosis of disease and a window of opportunity to detect the biological pathways amenable for preventive intervention.

Epigenetic regulation on left atrial function and disease recurrence after catheter ablation in atrial fibrillation

BackgroundGenetic variation and modifiable risk factors play a significant role in the pathogenesis of atrial fibrillation (AF). The influence of epigenetic modification on AF remains to be elucidated. We investigated the role of DNA methylation in the etiology of AF. Epigenetic evaluation was performed in 115 AF patients who underwent radiofrequency catheter ablation in a single institution. We measured methylation at approximately 850,000 bp cytosine-phosphate-guanine (CpG) sites in the 115 samples. The degree of methylation was compared across seven classification criteria: type of AF, late recurrence, impaired left atrium (LA) function, late gadolinium enhancement, LA diameter, LA volume, and flow velocity of the LA appendage.ResultsThe four most significantly methylated genes were DEFB104B, C3, TANC1, and TMEM9B. The DEFB104B gene (cg20223677 in the transcription start site), which encodes β-defensin 104B, was hypomethylated in three groups: AF patients with late recurrence, impaired LA function, and impaired LAA flow velocity. Enriched functional annotation of the differentially methylated datasets revealed that five out of the seven AF groups in this cohort were associated with genes involved in the cell movement of endothelial cell lines, sprouting angiogenesis by endothelial cell lines, or migration of endothelial cell lines.ConclusionsEpigenetic profiling revealed that epigenetic modification might affect important characteristics of AF. Our results suggest that the pathogenesis of AF might be affected by not only genetic variation or modifiable factors but also by epigenetic modulation.

IFN-γ-producing TH1 cells and dysfunctional regulatory T cells contribute to the pathogenesis of Sjögren's disease.

Sjögren's disease (SjD) is an autoimmune disorder characterized by progressive salivary and lacrimal gland dysfunction, inflammation, and destruction, as well as extraglandular manifestations. SjD is associated with autoreactive B and T cells, but its pathophysiology remains incompletely understood. Abnormalities in regulatory T (Treg) cells occur in several autoimmune diseases, but their role in SjD is ambiguous. We had previously shown that the function and development of Treg cells depend on store-operated Ca2+ entry (SOCE), which is mediated by ORAI1 Ca2+ channels and stromal interaction protein 1 (STIM1) and STIM2. Here, we show that mice with a Foxp3+ Treg cell-specific deletion of Stim1 and Stim2 develop a phenotype that fulfills all classification criteria of human SjD. Mutant mice have salivary and lacrimal gland inflammation characterized by strong lymphocyte infiltration and transcriptional signatures dominated by T helper 1 (TH1) and interferon (IFN) signaling. CD4+ T cells from mutant mice are sufficient to induce SjD-like disease in an IFN-γ-dependent manner. Inhibition of IFN signaling with the JAK1/2 inhibitor baricitinib alleviated CD4+ T cell-induced SjD in mice. These findings are consistent with the transcriptional profiles of CD4+ T cells from patients with SjD, which indicate enhanced TH1 but reduced memory Treg cell function. Together, our study provides evidence for a critical role of dysfunctional Treg cells and IFN-γ-producing TH1 cells in the pathogenesis of SjD.

Evaluating the Financial Performance of Colombian Companies: A Data Envelopment Analysis Without Explicit Inputs and Technique for Order Preference by Similarity to the Ideal Solution Approach

The evaluation and ranking of companies in any sector are generally based on a single measure of financial success, so the results obtained vary according to the classification criteria used. This study applies a multi-criteria approach to develop a classification of the largest companies in Colombia based on their financial results for the period 2022–2023. An analysis of 100 companies was conducted, utilizing four critical criteria: operating income, net profit, total assets, and equity. The evaluation followed a two-stage process. In the first stage, the weights or importance of each selected criterion were objectively established using data envelopment analysis without explicit inputs (DEA-WEIs). This approach reveals that operating income (35.23%) and total assets (28.57%) are the most influential criteria, while net profit is the least influential (13.51%). In the second stage, companies are ranked using the Technique for Order Preference by Similarity to the Ideal Solution (TOPSIS), with the results highlighting Refinería de Cartagena, Empresas Públicas de Medellín, and Terpel S.A. as the top-performing companies. The classification shows clear differentiation, forming two statistically distinct groups validated through discriminant analysis, achieving a 100% correct classification rate. These findings provide actionable insights for benchmarking and improving financial performance in the corporate sector.

Prevalence of Antiphospholipid Antibody Syndrome Among Patients with Recurrent Pregnancy Loss: Impact of the Revised 2023 ACR/EULAR Antiphospholipid Syndrome Criteria.

Objectives: Current guidelines recommend systematic screening for rheumatic diseases (RDs), including antiphospholipid syndrome (APS), in patients with recurrent pregnancy loss (RPL). However, these recommendations are based on limited evidence, as data on the prevalence of RD in this specific population remain scarce. In particular, the impact of the recent update to the ACR/EULAR classification criteria for APS on the prevalence of RD among RPL patients has yet to be clarified. To address these gaps, this study aims to (i) assess the impact of the 2023 ACR/EULAR APS classification criteria in patients with recurrent pregnancy loss (RPL); and (ii) analyze the prevalence of RD in these patients. Methods: We conducted a retrospective cohort study at Rennes University Hospital. From January 2010 to December 2021, all patients referred to the Clinical Immunology Department for RPL were included. Patients were eligible if they had undergone a full RPL evaluation, according to guidelines. Results: We included 165 women with RPL. APS according to the Sydney criteria was found in 24 (14.5%) patients. No significant differences in obstetric history or clinical signs were observed between APS-positive and APS-negative individuals. Only two patients fulfilled the updated 2023 APS criteria, resulting in 163 (98.8%) patients being classified as having unexplained recurrent pregnancy loss (uRPL). Among them, 108 had a new pregnancy following uRPL, resulting in 87 (81%) live births and 21 (19%) recurrent miscarriages. We did not identify any prognostic factor associated with subsequent pregnancy outcomes, including the patients' antiphospholipid biological profile. We found a prevalence of non-APS RD of only 2.4% in the study population, including systemic lupus erythematosus, rheumatoid arthritis, and Behçet's disease. Conclusions: APS was identified in 14.5% of the patients based on the former Sydney criteria and 1.2% according to the revised criteria. The lack of clinical differences between APS and non-APS patients aligns with previously reported limitations of the Sydney criteria in accurately identifying aPLA-related RPL. According to the rarity of APS as per the updated criteria, future large collaborative trials will be needed to further characterize APS-related RPL patients and to determine the best treatment strategy for future pregnancies.

China’s regulations on the attribution of AI-generated content: an exploration based on the open-ended approach

Abstract This article examines the issue of copyright ownership for AI-generated content under China’s current copyright law, with a particular focus on the open-ended classification system introduced by the 2020 Copyright Law amendment and its practical implications. This amendment marks a shift in Chinese copyright law from a closed-list approach to an open-ended approach, thereby extending copyright protection to non-traditional works, such as AI-generated content. Despite including catch-all provisions and refining the criteria for work classification, ambiguities persist regarding the requirements for originality and the categorical hierarchy, continuing to fuel debates on the copyrightability of AI-generated content. This article analyses representative copyright dispute cases involving AI-generated content to illustrate the adjudicative stance of Chinese courts. Significant differences exist among courts regarding standards for originality and intellectual achievement, with rulings often heavily reliant on individual judges’ interpretations of technology and the creative process, which results in considerable discretion. Additionally, the ambiguous relationship between traditional work categories and catch-all provisions facilitates the classification of AI-generated content as a new type of work, potentially leading to an undue expansion of copyright protection. This article offers recommendations from both legislative and judicial perspectives, proposing the establishment of a ‘priority application for traditional work categories’ principle in legislation, issuing judicial interpretations to clarify AI’s instrumental role in creation and advocating for a cautious and substantive assessment in court to determine whether AI-generated content meets the characteristics of a protected work.

Diagnosis and management of antiphospholipid syndrome.

Antiphospholipid syndrome is an autoimmune disease characterised by thrombotic and/or obstetric manifestations with persistent antiphospholipid antibodies. Diagnosis involves confirming the persistence of antiphospholipid antibodies in symptomatic patients, using validated classification criteria as a guide. The likelihood of obtaining false-positive or false-negative test results in certain settings, and the lack of standardisation between laboratory methods, are important considerations. Patients who have had thrombotic manifestations require lifelong anticoagulation from the first thrombotic event, typically with warfarin. Patients with a history of thrombotic and/or obstetric manifestations who become pregnant should receive low-molecular-weight heparin and low-dose aspirin during pregnancy and postpartum. Testing asymptomatic people is not recommended, except in the context of systemic lupus erythematosus. Management of asymptomatic people with persistent antiphospholipid antibodies depends on their individual antibody profile and risk factors.

Arthralgia and Extraintestinal Manifestations in Crohn's Disease Elevate the Risk of IBD-Related Arthritis over Sacroiliitis.

Inflammatory bowel disease (IBD) related arthritis is the most prevalent extraintestinal manifestation (EIM) of IBD, ranging between 10 and 39%. Magnetic resonance enterography (MRE) is used to assess small bowel disease involvement in Crohn's disease (CD) and can detect signs of sacroiliitis in up to 23.5% of patients. The predicting role of sacroiliitis detected on MRE is still unknown. The aim of this study is to evaluate the predictive role of sacroiliitis at MRE and other clinical features for IBD-related arthritis development in a cohort of adult patients with CD. Between December 2012 and May 2020, consecutive patients with CD who performed MRE were enrolled in the study. Patients with a previous diagnosis of IBD-related arthritis were excluded. A baseline demographics and clinical characteristics of the patients were retrospectively collected. The identification of new-onset IBD-related arthritis events during the follow-up was based on rheumatological clinical diagnosis and fulfillment of the ASAS classification criteria. Ninety-five patients, mean age 43.9 years (standard deviation [SD] ± 16.6), 52.6% female were enrolled in the study with a median follow-up of 83months (Q25:75 25:143). Six out 95 (6.3%) developed IBD-related arthritis with a mean time of 11months (SD ± 16.8). Sacroiliitis detected on MRE was not associated with an increased risk of IBD-related arthritis (odds ratio [OR] = 2.12 [95% confidence interval (CI) 0.36, 12.53, p = 0.408]). In contrast, the presence of arthralgia and EIMs were found to be a predictor for IBD-related arthritis development (OR = 84.0 [95% CI 8.18, 862.39, p < 0.0001] and OR = 7.37 [95% CI 1.25, 43.32, p = 0.027], respectively). This study highlights that sacroiliitis, as assessed by MRE, was not associated with the development of IBD-related arthritis, whereas extraintestinal manifestations and arthralgia were significantly associated with later IBD-related arthritis development in patients with CD.

Non-criteria antiphospholipid antibody profiles and thrombotic outcomes in a cohort of patients with systemic lupus erythematosus

ObjectivesAntiphospholipid syndrome (APS) is characterised by the presence of antiphospholipid antibodies (aPLs) and clinical outcomes of thrombosis and/or obstetric morbidity and is associated with systemic lupus erythematosus (SLE). IgG antiphosphatidylserine/prothrombin...

Incomplete lupus in adults. who, when, and how to treat?

Systemic lupus erythematosus (SLE) often evolves from a preclinical phase, characterized by immune dysregulation and incomplete clinical manifestations that do not meet classification criteria. This article explores the spectrum of incomplete lupus erythematosus (ILE) and its potential transition to classified SLE. Notably, a significant proportion of ILE patients exhibit various clinical manifestations, including autoimmune abnormalities and organ damage, despite not fulfilling formal SLE criteria. Recent studies suggest that 10–50% of ILE cases may progress to SLE within five years, often presenting a milder form without severe organ involvement. The article highlights the need for improved screening and monitoring strategies for people with a high risk of SLE, particularly those with risk factors or serological abnormalities. Current guidelines lack comprehensive management approaches for ILE patients either symptomatic or asymptomatic, emphasizing the necessity for further research into preventive therapies, such as hydroxychloroquine, to mitigate disease progression. Ultimately, understanding the pathogenesis and clinical management of incomplete forms of SLE is crucial for early intervention and improved patient outcomes.

Impact of the 2023 ACR/EULAR Classification Criteria on START2 Antiphospholipid Registry.

The recently published ACR/EULAR classification criteria score (3 points or more) both clinical and laboratory criteria to define the presence of antiphospholipid syndrome (APS). The clinical criteria have been better defined while laboratory criteria remain the same [lupus anticoagulant (LA), anticardiolipin (aCL) and anti ß2-Glycoprotein I (aß2GPI) antibodies] but with different impact (points) on the classification of patients. APS is excluded if more than 3 years separate positive test for antiphospholipid antibodies (aPL) and clinical manifestation. The present study evaluates how many patients would be excluded by the new criteria among those enrolled as APS in the START 2 antiphospholipid registry. The analysis includes 380 patients (274 APS and 106 carriers). Of 274 patients classified as APS, 118 (43%) did not match the new ACR/EULAR criteria for various reasons. First, the determination of aCL and aß2GPI antibodies was performed by automated instrumentations not allowed in the new criteria. Second, laboratory test score was less than 3 and this was due to an isolated IgM aCL or IgM aß2GPI in most cases and to isolated LA unconfirmed after 12 weeks in few cases. Third, 2 patients had a positive laboratory tests more than 3 years after the clinical event. Of the 106 carriers, 62% had aCL and aß2GPI determined by ELISA thus meeting the ACL/EULAR laboratory criteria but were negative for clinical criteria. This study shows that many patients classified as APS in the START 2 registry do not match the classification using the new ACR/EULAR criteria.

The first all-sky survey of star-forming galaxies with eROSITA. Scaling relations and a population of X-ray luminous starbursts

In this work, we present the results from a study of X-ray normal galaxies, that is, galaxies not harbouring active galactic nuclei (AGN), using data from the first complete all-sky scan of the X-ray survey (eRASS1) obtained with eROSITA on board the Spectrum-Roentgen-Gamma observatory. eRASS1 provides the first unbiased X-ray census of local normal galaxies, thus allowing us to study the X-ray emission (0.2-8.0 ) from X-ray binaries (XRBs) and the hot interstellar medium in the full range of stellar population parameters present in the local Universe. By combining the updated version of the Heraklion Extragalactic Catalogue (HECATE v2.0) value-added catalogue of nearby galaxies (Distance ; lesssim \,200$Mpc) with the X-ray data obtained from eRASS1, we studied the integrated X-ray emission from normal galaxies as a function of their star-formation rate ( ), stellar mass ( ), metallicity, and stellar population age. After applying stringent optical and mid-infrared activity classification criteria, we constructed a sample of 18790 bona fide star-forming galaxies (HEC-eR1 galaxy sample) with measurements of their integrated X-ray luminosity (using each galaxy's D$_ $) over the full range of stellar population parameters present in the local Universe. By stacking the X-ray data in bins, we studied the correlation between the average X-ray luminosity and the average stellar population parameters. We also present updated and -metallicity scaling relations based on a completely blind galaxy sample and accounting for the scatter dependence on the The average X-ray spectrum of star-forming galaxies is well described by a power law ($ $) and a thermal plasma component ($kT = 0.70^ $). We find that the integrated X-ray luminosity of the individual HEC-eR1 star-forming galaxies is significantly elevated (reaching $10^ $) with respect to what is expected from the current standard scaling relations. The observed scatter is also significantly larger. This excess persists even when we measured the average luminosity of galaxies in and metallicity bins, and it is stronger (up to $ sim 2$ dex) towards lower s. Our analysis shows that the excess is not the result of the contribution by hot gas, low-mass XRBs, background AGN, low-luminosity AGN (including tidal disruption events), or stochastic sampling of the XRB X-ray luminosity function. We find that while the excess is generally correlated with lower metallicity galaxies, its primary driver is the age of the stellar populations. Our analysis reveals a sub-population of very X-ray luminous starburst galaxies with higher specific SFRs (sSFRs), lower metallicities, and younger stellar populations. This population drives upwards the X-ray scaling relations for star-forming galaxies and has important implications for understanding the population of XRBs contributing in the most X-ray luminous galaxies in the local and high-redshift Universe. These results demonstrate the power of large blind surveys such eRASS1, which can provide a more complete picture of the X-ray emitting galaxy population and their diversity, revealing rare populations of objects and recovering unbiased underlying correlations.

ИНТЕГРАЦИЯ КАЗАХСТАНСКОГО КОДЕКСА ПУБЛИЧНОЙ ОТЧЕТНОСТИ KAZRC ДЛЯ ОЦЕНКИ РЕСУРСОВ МЕСТОРОЖДЕНИЙ АККАРГИНСКОГО РУДНОГО ПОЛЯ

The presented article covers the issues of transition of geological reporting from GKZ standards to international KAZRC standards, in relation to gold reserves of deposits of the Akkarga ore field. The fundamental differences in calculating reserves using the old and new systems, introduced from 2024, are considered. When carrying out the study, a brief description of the deposit, the methodology of the geological exploration work, and the technology of mining and processing of ores for rapid and visual data analysis were considered. A classification of ore reserves was given according to the previously used GKZ system and the new KAZRC system. The criteria for the international classification of resources and the criteria for the classification of reserves (GKZ) are analyzed and described in detail. In order to show the difference between these two systems, reserves were calculated and the mineral resources of the Akkarga ore field deposits were assessed. The results of calculating reserves showed that depending on the method used, the amount of ore and metal differs significantly. New methods for data verification are also described. Procedures are described that ensure the quality, accuracy, reliability and transparency of analytical information. New approaches to visualization and interpretation of the obtained results of quality control of geological exploration are also presented. Modifying factors are considered, including mining, technological, metallurgical, infrastructural, economic, marketing, environmental, social and administrative aspects of the Akkarginskoye deposit, used in international KAZRC standards, in the classification and assessment of mineral resources. They have shown their effectiveness in assessing risks for subsoil users. All this as a whole gives us an idea and understanding of the differences in the approaches used in assessing mineral reserves and resources.

Increased Risk of Myositis-Specific and Myositis-Associated Autoantibodies After COVID-19 Pandemic and Vaccination: A Spanish Multicenter Collaborative Study.

Background: Emerging evidence suggests that SARS-CoV-2 infection and vaccines may trigger autoimmune responses in predisposed individuals. Idiopathic inflammatory myopathies (IIMs) are diseases with diverse clinical manifestations, often associated with myositis autoantibodies (MAs). Diagnosing IIM is challenging due to limitations in classification criteria and diagnostic assays. This study aimed to describe the incidence of IIM following SARS-CoV-2 infection or vaccination and compare rates between exposures. Methods: A multicenter observational study was conducted with 788 patients from 11 Spanish referral centers. A total of 1209 autoantibodies including myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs), were analyzed using line blot immunoassay (LIA). Results: The study identified distinct patterns in aminoacyl-tRNA synthetase (ARS) antibody frequencies compared to pre-pandemic periods. Anti-PL-7 was the most prevalent ARS antibody (14.85%), while anti-Jo-1 was less frequent (7.23%). Anti-MDA5, commonly linked to SARS-CoV-2 infection, was detected in 11.68%. ANA positivity was observed in 60.66%, suggesting an autoimmune background. The most frequent diagnoses were anti-synthetase syndrome (ASSD) or IIM-non-ASSD (21.31%), followed by other systemic autoimmune diseases (SAIDs) (13.57%). Among the cohort, 91.13% received at least one dose of a messenger RNA (mRNA) COVID-19 vaccine, with a median of three doses per patient. Patients with prior SARS-CoV-2 infection or heterologous vaccination showed a higher frequency of multiple autoantibody positivity (p < 0.05), reflecting distinct immune signatures. Conclusions: This study provides valuable insights into the autoimmune risks and phenotypes associated with SARS-CoV-2 infection and vaccination, establishing a basis for further research on IIM and its link to MSAs and MAAs.

History of antineutrophil cytoplasmic autoantibodies : Milestones in rheumatology.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are autoimmune inflammatory small-vessel disorders with potentially life-threatening organ manifestations. Recent disease definitions and classification criteria allow distinction between granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and non-granulomatous microscopic polyangiitis (MPA). The discovery of ANCA-autoantibodies directed against proteolytic enzymes of neutrophil granules-has enabled earlier diagnosis of AAV and paved the way to stage-adapted treatments. ANCA testing initially relied on different immunofluorescence patterns, i.e., cytoplasmic ANCA (C-ANCA) vs. perinuclear ANCA (P-ANCA), in ethanol-fixed neutrophils. This is nowadays outperformed by well-standardized immunoassays against the ANCA target antigens proteinase3 (PR3) and myeloperoxidase (MPO) for the diagnosis of small-vessel vasculitides. The discovery of ANCA has contributed substantially to unravelling the pathogenesis of AAV, which comprises neutrophil degranulation, NETosis with concurrently amplified ANCA antigen presentation, and intra- and transmural vascular inflammation involving the alternative complement system in susceptible individuals. There is agenetic disposition concerning certain HLA alleles and polymorphisms of the proteinase3 gene. Furthermore, epigenetic modifications impact on disease activity and relapse. During follow-up, the ANCA titer is not areliable mirror of disease activity; however, PR3-ANCA positivity is associated with agreater likelihood of relapse and abetter treatment response to rituximab as compared to cyclophosphamide/azathioprine. Within the past 60years, the discovery of ANCA has revolutionized the ability to diagnose, understand, classify, and treat AAV in atargeted manner.

Генотип-фенотипическая характеристика детей с семейной средиземноморской лихорадкой (периодической болезнью) в регионе Российской Федерации

Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease (AID) caused by mutations in the MEFV gene. The purpose of this research was to investigate clinical variability and potential genotypic and phenotypic correlations in children with clinically verified FMF and MEFV gene mutations in the City of Moscow, Russia. Materials and methods used: among 188 pediatric patients with suspected monogenic AIDs, MEFV gene mutations were identified in 9 through sequencing. In order to confirm the FMF diagnosis, patients were evaluated according to the modified Tel-Hashomer criteria and the Eurofever/PRINTO classification criteria. Results: clinical picture of FMF was characterized by the presence of periodic attacks of the disease lasting from 1 to 7 days accompanied by fever and increased acute phase reactants (observed in all patients), abdominal pain (in 8 children), peritonitis requiring diagnostic laparotomy (in a single child), chest pain (in 2 patients), pleurisy (in a single child), arthritis (in 7 children) and erysipeloid-like rash (in a single child). The pathogenic mutation M694V was identified in the MEFV gene in 8 out of 9 patients, this mutation was homozygous in 5 patients and heterozygous in 1, M680I mutation was detected on another allele in 2 children (compound heterozygous), and a mutation of unknown significance K695R was detected in a single child. No obvious clinical differences were found between homozygotes and heterozygotes as well as compound heterozygotes. Clinical response was achieved in 5 patients with colchicine monotherapy while canakinumab was prescribed to 4 patients due to ongoing attacks. Conclusion: the diagnosis involving the identification of MEFV gene mutations may allow the assessment of genotype-phenotype correlations in patients with FMF.

Characteristics That Predict Psoriatic Arthritis by the Classification Criteria for Psoriatic Arthritis in Patients With Juvenile Idiopathic Arthritis 18 Years After Disease Onset.

The purposes of this study were to assess the clinical characteristics of patients with juvenile idiopathic arthritis (JIA) who fulfill the ClASsification criteria for Psoriatic ARthritis (CASPAR) 18 years after disease onset in a population-based setting and to identify features likely to predict psoriatic arthritis (PsA). Patients with JIA from defined geographic regions of Denmark, Finland, Norway, and Sweden with disease onset from 1997 to 2000 were enrolled prospectively and followed up for 18 years. Clinical, laboratory, and heredity data for psoriasis were collected. Patients were classified according to the International League of Associations for Rheumatology (ILAR) criteria at baseline, and we applied ILAR and CASPAR criteria at 18 years. Logistic regression was performed to study the effects of JIA-related characteristics and heredity for psoriasis on being classified for PsA. Among the 510 patients enrolled, 434 participated in the 18-year follow-up, 28 (6.5%) met the ILAR criteria, and 41 (9.4%) fulfilled the CASPAR criteria. Patients with wrist or subtalar joint involvement at onset had higher odds of being classified with PsA at 18 years (odds ratio [OR] 3.3, P = 0.02 and OR 12.9, P = 0.01, respectively). Presence of psoriasis, nail abnormalities, or dactylitis showed significant association with development of PsA (OR 20.2, P < 0.001; OR 11.6, P = 0.002; and OR 43.4, P < 0.001, respectively). CASPAR criteria identify more patients with PsA compared with ILAR criteria and may better capture the heterogeneous nature of the disease. Presence of psoriasis and dactylitis at disease onset were the strongest predictors for the development of PsA. Further studies on the utility of CASPAR criteria in patients with JIA are needed.