Publications by authors named "Tadej Avcin"

Systemic Lupus Erythematosus (SLE) is a rare, heterogeneous, potentially life-threatening autoimmune disease. Presence of kidney or other major organ (brain, heart or lung) involvement are predictors of poor outcome and in a subset of patients resistant to 1st or 2nd line conventional treatment. The 10-year mortality remains around 10-15 %.

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  • Some experts and patients worked together to create a set of important data to help study childhood lupus, a disease that affects children.
  • They made two datasets: a Core Dataset with 46 necessary items and an Expanded Dataset with 26 extra items, to help gather more information.
  • This new information will help researchers around the world study childhood lupus better by using the same important facts and details.
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  • Advanced chronic lymphocytic leukemia (CLL) leads to an increase in regulatory T cells (Tregs) and higher risk of severe infections, particularly linked to heightened FOXP3 expression in these cells.
  • A study utilizing flow cytometry assessed STAT5 signaling in Treg subsets among 37 CLL patients, looking at how this related to disease progression and tumor mass.
  • Findings indicated that a specific subset of activated Tregs (aTregs) was correlated with advanced disease and severe infection rates, suggesting aTreg levels could serve as a marker for disease severity and that increased STAT5 signaling may contribute to their expansion.
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Objectives: (1) characterizing a group of spondyloarthritis (SpA) patients with systemic auto-inflammatory symptoms (S-SpA); (2) comparing SpA features with and without auto-inflammatory symptoms; (3) comparing the auto-inflammatory features of S-SpA and Still's disease (SD).

Methods: Retrospective observational study. Clinical data of adult and pediatric patients with S-SpA, SD or SpA were collected retrospectively and analyzed.

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  • The article discusses a long-term study on the safety and effectiveness of etanercept, a treatment for juvenile idiopathic arthritis (JIA), in children and young adults over a span of ten years.
  • JIA is an inflammatory condition that causes joint pain and stiffness, usually diagnosed before age 16, requiring ongoing treatment until the disease is in remission.
  • The research highlights the need to evaluate the long-lasting safety of etanercept, which modulates the immune system to alleviate symptoms of arthritis.
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Antiphospholipid syndrome in children.

Best Pract Res Clin Rheumatol

September 2024

Article Synopsis
  • Antiphospholipid syndrome (APS) is a rare but severe disease in children, leading to frequent and serious complications compared to adults, including higher instances of thrombotic events and life-threatening episodes.
  • * Pediatric patients often experience nonthrombotic symptoms that can occur before any blood clotting events.
  • * The review discusses new classification criteria, emerging treatments like B cell and complement inhibitors, and APS cases in infants linked to maternal aPL, highlighting long-term neurodevelopmental issues rather than immediate thrombotic complications.
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  • The 2023 ACR/EULAR antiphospholipid syndrome (APS) classification criteria development used a four-phase methodology to identify high likelihood patients for research purposes.
  • In the final phase, a multicriteria decision analysis (MCDA) helped rank the importance of candidate criteria based on evaluations from 192 real-world patients suspected of having APS.
  • The consensus reached emphasized the need for separate clinical and laboratory scores for APS classification, aiming for greater specificity compared to existing systems that rely on a single score.
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  • Paediatric patients with autoimmune rheumatic diseases (pARD) are at a higher risk for severe infections, including COVID-19, so timely vaccination with the BNT162b2 vaccine was prioritized.
  • The study compared the immune response (antibody levels) among three groups: those who had COVID-19, those who were vaccinated, and those who experienced both; data was gathered from March 2020 to April 2022.
  • Results showed that antibody responses were stronger in vaccinated individuals and those who experienced both the vaccine and infection, and the vaccine had a good safety profile, with most vaccinated pARD remaining asymptomatic for COVID-19 over a 41.3-week observation period.
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Maternal autoimmune rheumatic diseases can influence the outcomes of children through several life stages. During pregnancy, maternal inflammation and autoantibodies can hinder fetal development and lead to growth restriction, preterm birth, and low birth weight; prematurity, especially at extreme gestational ages, can in turn impair future child health. Treatment with compatible immunomodulatory drugs and preventive medications aims to keep maternal disease under control and minimise the risk of adverse pregnancy outcomes.

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Objectives: The aim of the study was to establish an international multicenter registry to collect data on patients with Multisystem Inflammatory Syndrome in Children (MIS-C), in order to highlight a relationship between clinical presentation, age of onset and geographical distribution on the clinical outcome.

Study Design: Multicenter retrospective study involving different international societies for rare immunological disorders.1009 patients diagnosed with MIS-C between March and September 2022, from 48 centers and 22 countries were collected.

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Aim: To comprehensively review the literature on multisystem inflammatory syndrome in children (MIS-C).

Methods: Narrative review of relevant studies published between April 2020 and January 2024.

Results: MIS-C is a SARS-CoV-2-related hyperinflammatory syndrome developing 2-6 weeks after COVID-19 in genetically susceptible individuals.

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Introduction: This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD).

Methods: This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled.

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Background: To evaluate long-term outcomes and prognostic factors in patients with juvenile idiopathic arthritis (JIA), presenting as oligoarthritis, who received IAC as the first treatment for their disease.

Methods: We conducted retrospective study at the University Children's Hospital Ljubljana, Slovenia, from January 2015 to May 2023 in children with JIA, clinically presenting as oligoarthritis receiving intra-articular corticosteroid injection (IAC) as the initial treatment. Patient and treatment data were collected, and the outcomes were categorized into three groups based on the later need for therapy: no therapy needed, only additional IAC needed and systemic therapy needed.

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Background: Data suggest that food allergies greatly impact a child's health and growth due to inadequate nutrient intake. Our study aimed to establish the long-term outcome of children with food allergies compared to a control group.

Methods: This study was a retrospective cohort study with longitudinal follow-up with a mean period of 4.

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Introduction: Much has been written and spoken about telemedicine since about two decades including an article in this journal at the start of the pandemic. It took a global catastrophe to enforce its usage across the world in various medical specialties. Telemedicine however remains unstructured, unregulated and lacks uniformity.

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In recent years, imaging has become increasingly important to confirm diagnosis, monitor disease activity, and predict disease course and outcome in children with juvenile idiopathic arthritis (JIA). Over the past few decades, great efforts have been made to improve the quality of diagnostic imaging and to reach a consensus on which methods and scoring systems to use. However, there are still some critical issues, and the diagnosis, course, and management of JIA are closely related to clinical assessment.

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Objectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis.

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Objective: To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR.

Methods: This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and literature review; (2) Phase II, criteria reduction by modified Delphi and nominal group technique exercises; (3) Phase III, criteria definition, further reduction with the guidance of real-world patient scenarios, and weighting via consensus-based multicriteria decision analysis, and threshold identification; and (4) Phase IV, validation using independent adjudicators' consensus as the gold standard.

Results: The 2023 ACR/EULAR APS classification criteria include an entry criterion of at least one positive antiphospholipid antibody (aPL) test within 3 years of identification of an aPL-associated clinical criterion, followed by additive weighted criteria (score range 1-7 points each) clustered into six clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and two laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti-β-glycoprotein I antibodies).

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Article Synopsis
  • - The study aimed to create new and more specific classification criteria for antiphospholipid syndrome (APS) in collaboration with the American College of Rheumatology (ACR) and EULAR, using a detailed four-phase methodology.
  • - The new criteria require at least one positive antiphospholipid antibody test and assign points across six clinical and two laboratory domains, classifying patients with a minimum of 3 points in both areas as having APS.
  • - Compared to the older Sapporo criteria, the 2023 ACR/EULAR criteria showed a significant increase in specificity (99% vs. 86%) but slightly lower sensitivity (84% vs. 99%), demonstrating a more refined approach to diagnosing APS.
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Objective: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis.

Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months.

Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males.

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Background: Paediatric patients with autoimmune rheumatic diseases (pARD) are often immunocompromised because of the disease and/or the therapy they receive. At the beginning of COVID-19 pandemic there was a great concern about the possibility of severe SARS-CoV-2 infection in these patients. The best method of protection is vaccination, so as soon as vaccine was licenced, we aimed to vaccinate them.

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Background: Postgraduate rheumatology training programmes are already established at a national level in most European countries. However, previous work has highlighted a substantial level of heterogeneity in the organisation and, in part, content of programmes.

Objective: To define competences and standards of knowledge, skills and professional behaviours required for the training of rheumatologists.

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