Log in

Relevant bibliographies by topics / SJIA / Journal articles

To see the other types of publications on this topic, follow the link: SJIA.

Journal articles on the topic 'SJIA'

Author: Grafiati

Published: 14 March 2023

Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles

Select a source type:

Consult the top 50 journal articles for your research on the topic 'SJIA.'

Next to every source in the list of references, there is an 'Add to bibliography' button. Press on it, and we will generate automatically the bibliographic reference to the chosen work in the citation style you need: APA, MLA, Harvard, Chicago, Vancouver, etc.

You can also download the full text of the academic publication as pdf and read online its abstract whenever available in the metadata.

Browse journal articles on a wide variety of disciplines and organise your bibliography correctly.

1

Ombrello, Michael J., Victoria L. Arthur, Elaine F. Remmers, et al. "Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications." Annals of the Rheumatic Diseases 76, no. 5 (2016): 906–13. http://dx.doi.org/10.1136/annrheumdis-2016-210324.

Full text

Abstract:

ObjectivesJuvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterised by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However, approximately half of children with sJIA develop destructive, long-standing arthritis that appears similar to other forms of JIA. Using genomic approaches, we sought to gain novel insights into

APA, Harvard, Vancouver, ISO, and other styles

2

Kim, Daeun, Jaeseung Song, Sora Lee, Junghyun Jung, and Wonhee Jang. "An Integrative Transcriptomic Analysis of Systemic Juvenile Idiopathic Arthritis for Identifying Potential Genetic Markers and Drug Candidates." International Journal of Molecular Sciences 22, no. 2 (2021): 712. http://dx.doi.org/10.3390/ijms22020712.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) is a rare subtype of juvenile idiopathic arthritis, whose clinical features are systemic fever and rash accompanied by painful joints and inflammation. Even though sJIA has been reported to be an autoinflammatory disorder, its exact pathogenesis remains unclear. In this study, we integrated a meta-analysis with a weighted gene co-expression network analysis (WGCNA) using 5 microarray datasets and an RNA sequencing dataset to understand the interconnection of susceptibility genes for sJIA. Using the integrative analysis, we identified a robust sJIA

APA, Harvard, Vancouver, ISO, and other styles

3

Ombrello, Michael J., Elaine F. Remmers, Ioanna Tachmazidou, et al. "HLA-DRB1*11 and variants of the MHC class II locus are strong risk factors for systemic juvenile idiopathic arthritis." Proceedings of the National Academy of Sciences 112, no. 52 (2015): 15970–75. http://dx.doi.org/10.1073/pnas.1520779112.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) is an often severe, potentially life-threatening childhood inflammatory disease, the pathophysiology of which is poorly understood. To determine whether genetic variation within the MHC locus on chromosome 6 influences sJIA susceptibility, we performed an association study of 982 children with sJIA and 8,010 healthy control subjects from nine countries. Using meta-analysis of directly observed and imputed SNP genotypes and imputed classic HLA types, we identified the MHC locus as a bona fide susceptibility locus with effects on sJIA risk that trans

APA, Harvard, Vancouver, ISO, and other styles

4

Kaleda, M., E. Fedorov, I. Nikishina, et al. "AB1252 RETROSPECTIVE SINGLE CENTER STUDY OF THE PREDICTIVE ROLE OF CERTAIN BIOMARKERS IN MACROPHAGE ACTIVATION SYNDROME RELATED TO SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS." Annals of the Rheumatic Diseases 81, Suppl 1 (2022): 1736.2–1737. http://dx.doi.org/10.1136/annrheumdis-2022-eular.3328.

Full text

Abstract:

BackgroundSystemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with signs of autoinflammation and high risk of macrophage activation syndrome (MAS). Immunopathogenesis of sJIA is based on excessive activation of innate immunity, which is characterized by hyperproduction of pro-inflammatory cytokines - interleukin (IL)-1 and IL-18, which play a key role in induction of synthesis of other pro-inflammatory mediators. Macrophage activation mediates the evidence of hyperferritinemia, which stimulates a pathological immune response with the development of MAS. In clinical practice,

APA, Harvard, Vancouver, ISO, and other styles

5

Rolfes, Elisabeth, Sae Lim von Stuckrad, and Tilmann Kallinich. "Eine neue Lungenerkrankung bei Kindern mit systemischer JIA/Still-Syndrom." Kinder- und Jugendmedizin 21, no. 05 (2021): 358–63. http://dx.doi.org/10.1055/a-1558-7356.

Full text

Abstract:

ZUSAMMENFASSUNGIm letzten Jahrzehnt hat eine neue Komplikation der systemischen juvenilen Arthritis mehr und mehr Beachtung in Fachkreisen und als „sJIA Lung Disease“ (sJIA-LD) Einzug in die Literatur gefunden. Die Kinder mit sJIA-LD präsentieren sich mit initial oft unspezifischen respiratorischen Symptomen, Hypoxie und Hautausschlag. Ein häufiges eindrückliches erstes Zeichen sind Trommelschlegelfinger mit digitalen Erythemen. Möglicherweise scheint die sJIA-LD gehäuft aufzutreten, wenn Kinder ein junges Alter bei sJIA-Diagnose hatten sowie ein oder mehrere Makrophagen-Aktivierungssyndrome i

APA, Harvard, Vancouver, ISO, and other styles

6

Krekhova, E. A., E. I. Alekseeva, and T. M. Dvoryakovskaya. "Predictors of response to therapy with biologicals in children with systemic juvenile idiopathic arthritis." Voprosy praktičeskoj pediatrii 16, no. 1 (2021): 64–78. http://dx.doi.org/10.20953/1817-7646-2021-1-64-78.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) is a severe orphan autoimmune disease characterized by fever, polymorphic skin rash, hepatosplenomegaly, serositis, generalized lymphadenopathy, and destructive arthritis. Proinflammatory cytokines, including interleukin-1 (IL-1), IL-6, IL-17, IL-18, and tumor necrosis factor (TNF), play a key role in sJIA pathogenesis. sJIA is refractory to conventional immunosuppressive antirheumatic drugs. Before the development of biologicals, sJIA patients had to receive corticosteroids constantly. The implementation of biologicals, such as tocilizumab (IL-6 i

APA, Harvard, Vancouver, ISO, and other styles

7

Vankova, D. D., E. I. Alekseeva, T. M. Dvoryakovskaya, et al. "Activity of systemic juvenile idiopathic arthritis in children immunized with pneumococcal 13-valent conjugate vaccine: prospective cohort study." Voprosy praktičeskoj pediatrii 15, no. 5 (2020): 40–50. http://dx.doi.org/10.20953/1817-7646-2020-5-40-50.

Full text

Abstract:

Evaluation of the safety profile of vaccines in patients with rheumatic diseases requires an assessment of their impact on disease activity. The effect of antipneumococcal vaccination on the activity of systemic juvenile idiopathic arthritis (sJIA) has not been studied so far. Objective. To evaluate the dynamics of sJIA activity after immunization with pneumococcal 13-valent conjugate vaccine (PCV13) of patients receiving biologicals. Patients and methods. This study included patients with sJIA in remission or active disease receiving biologicals during inpatient treatment and vaccinated with

APA, Harvard, Vancouver, ISO, and other styles

8

Zhou, Mi, Ruru Guo, Yong-Fei Wang, Wanling Yang, Rongxiu Li, and Liangjing Lu. "Application of Weighted Gene Coexpression Network Analysis to Identify Key Modules and Hub Genes in Systemic Juvenile Idiopathic Arthritis." BioMed Research International 2021 (August 13, 2021): 1–13. http://dx.doi.org/10.1155/2021/9957569.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) is a severe autoinflammatory disorder with a still not clearly defined molecular mechanism. To better understand the disease, we used scattered datasets from public domains and performed a weighted gene coexpression network analysis (WGCNA) to identify key modules and hub genes underlying sJIA pathogenesis. Two gene expression datasets, GSE7753 and GSE13501, were used to construct the WGCNA. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analyses were applied to the genes and hub genes in the sJIA modules. Cytoscap

APA, Harvard, Vancouver, ISO, and other styles

9

Sun, Juan, Miao Feng, Fengqi Wu, et al. "Plasma miR-26a as a Diagnostic Biomarker Regulates Cytokine Expression in Systemic Juvenile Idiopathic Arthritis." Journal of Rheumatology 43, no. 8 (2016): 1607–14. http://dx.doi.org/10.3899/jrheum.150593.

Full text

Abstract:

Objective.We sought to identify specific microRNA (miRNA) for systemic juvenile idiopathic arthritis (sJIA) and to determine the involvement of these miRNA in regulating the expression of cytokines.Methods.Microarray profiling was performed to identify differentially expressed miRNA in sJIA plasma. Levels of candidate miRNA and mRNA were assessed by real-time PCR, and cytokines were measured by ELISA. Dual-luciferase reporter assay was used to validate the direct interaction between miR-26a and interleukin 6 (IL-6).Results.Forty-eight miRNA were differentially expressed in the plasma of patien

APA, Harvard, Vancouver, ISO, and other styles

10

Gohar, Faekah, Angela McArdle, Melissa Jones, et al. "Molecular signature characterisation of different inflammatory phenotypes of systemic juvenile idiopathic arthritis." Annals of the Rheumatic Diseases 78, no. 8 (2019): 1107–13. http://dx.doi.org/10.1136/annrheumdis-2019-215051.

Full text

Abstract:

ObjectivesThe International League of Associations for Rheumatology classification criteria define systemic juvenile idiopathic arthritis (SJIA) by the presence of fever, rash and chronic arthritis. Recent initiatives to revise current criteria recognise that a lack of arthritis complicates making the diagnosis early, while later a subgroup of patients develops aggressive joint disease. The proposed biphasic model of SJIA also implies a ‘window of opportunity’ to abrogate the development of chronic arthritis. We aimed to identify novel SJIA biomarkers during different disease phases.MethodsChi

APA, Harvard, Vancouver, ISO, and other styles

11

Weaver, L. K. "Combining multiple biomarkers differentiates between active SJIA, SJIA-MAS and EBV-HLH." Clinical & Experimental Immunology 191, no. 3 (2017): 253–54. http://dx.doi.org/10.1111/cei.13063.

Full text

APA, Harvard, Vancouver, ISO, and other styles

12

Gurion, R., T. J. A. Lehman, and L. N. Moorthy. "Systemic Arthritis in Children: A Review of Clinical Presentation and Treatment." International Journal of Inflammation 2012 (2012): 1–16. http://dx.doi.org/10.1155/2012/271569.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. Despite being grouped under JIA, it is considered to be a multifactorial autoinflammatory disease. The objective of this paper is to review the epidemiology, pathogenesis, genetics, clinical manifestations, complications, therapy, prognosis, and outcome of sJIA. The presentation and clinical manifestations of sJIA have not changed much in the past several decades, but the collective understanding of the pathogenesis and the develop

APA, Harvard, Vancouver, ISO, and other styles

13

Quartier, Pierre. "Systemic Juvenile Idiopathic Arthritis/Pediatric Still’s Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches." Journal of Clinical Medicine 11, no. 5 (2022): 1357. http://dx.doi.org/10.3390/jcm11051357.

Full text

Abstract:

Background: Systemic Juvenile Idiopathic Arthritis (SJIA)/Pediatric Still’s disease is associated with different phenotypes and outcomes from currently available treatments. Methods: A review of opinion, based on personal experience in a reference pediatric rheumatology center and key publications, to explore the most important questions regarding disease heterogeneity and treatment approaches. Results: A few situations deserve particular attention: 1/patients with recent-onset SJIA who may benefit from a treat-to-target approach with a key place for interleukin (IL)-1 inhibition; 2/SJIA patie

APA, Harvard, Vancouver, ISO, and other styles

14

Davidson, Nicole, Hemalatha G. Rangarajan, Kyla Driest, Rajinder P. S. Bajwa, Veronika Polishchuk, and Rolla F. Abu-Arja. "Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome." Case Reports in Rheumatology 2021 (May 24, 2021): 1–3. http://dx.doi.org/10.1155/2021/9323141.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) is characterized by arthritis, fever, rash, lymphadenopathy, hepatosplenomegaly, and serositis. Macrophage activation syndrome is the most feared complication of sJIA with a high risk of mortality. We report a 16-year-old female diagnosed with refractory systemic juvenile idiopathic arthritis (sJIA) complicated by recurrent macrophage activation syndrome (MAS), severe joint disease, and lung involvement requiring prolonged immunosuppressive therapy. She received a matched unrelated allogeneic hematopoietic cell transplant (Allo-HCT) using a reduced

APA, Harvard, Vancouver, ISO, and other styles

15

Ishikawa, S., T. Mima, C. Aoki, et al. "Abnormal expression of the genes involved in cytokine networks and mitochondrial function in systemic juvenile idiopathic arthritis identified by DNA microarray analysis." Annals of the Rheumatic Diseases 68, no. 2 (2008): 264–72. http://dx.doi.org/10.1136/ard.2007.079533.

Full text

Abstract:

Objectives:Systemic juvenile idiopathic arthritis (sJIA) is a rheumatic disease in childhood characterised by systemic symptoms and a relatively poor prognosis. Peripheral leukocytes are thought to play a pathological role in sJIA although the exact cause of the disease is still obscure. In this study, we aimed to clarify cellular functional abnormalities in sJIA.Methods:We analysed the gene expression profile in peripheral leukocytes from 51 patients with sJIA, 6 patients with polyarticular type JIA (polyJIA) and 8 healthy children utilising DNA microarrays. Gene ontology analysis and network

APA, Harvard, Vancouver, ISO, and other styles

16

Villarreal, M. G., S. Bonaldi, L. Perez, and M. Katsikas. "AB1007 SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS: ARE DIFFERENT CLINICAL PATTERNS ASSOCIATED WITH S100A8/S100A9 SERUM LEVELS?" Annals of the Rheumatic Diseases 79, Suppl 1 (2020): 1797.1–1797. http://dx.doi.org/10.1136/annrheumdis-2020-eular.5094.

Full text

Abstract:

Background:Systemic juvenile idiopathic arthritis (SJIA) is a category of Juvenile Idiopathic Arthritis (JIA). Different clinical patterns (articular/systemic/both of them combined) have been recognized, possibly identifying distinct subpopulations. Serum biomarkers that reflect disease activity include S100A8/S100A9 (S100A8/9), however to date patterns of SJIA and their association with S100A8/9 has not been tested.Objectives:To evaluate S100A8/9 levels in a cohort of patients with SJIA. To determinate S100A8/9 inactive vs inactive visits. To distinguish patterns on SJIA and their association

APA, Harvard, Vancouver, ISO, and other styles

17

Rossi-Semerano, Linda, and Isabelle Koné-Paut. "Is Still's Disease an Autoinflammatory Syndrome?" International Journal of Inflammation 2012 (2012): 1–5. http://dx.doi.org/10.1155/2012/480373.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA), formerly called Still's disease, is officially classified as a subset of juvenile idiopathic arthritis (JIA). Beside arthritis, it is characterized by prominent systemic features and a marked inflammatory response. Even if it is still included in the group of juvenile arthritides, sJIA is set apart from all the other forms of JIA. This disorder has markedly distinct clinical and laboratory features suggesting a different pathogenesis. sJIA does not show any association with HLA genes or with autoantibodies and is characterised by an uncontrolled a

APA, Harvard, Vancouver, ISO, and other styles

18

Macaubas, Claudia, Khoa Nguyen, Ariana Peck, et al. "Monocyte phenotypes in systemic juvenile idiopathic arthritis (44.13)." Journal of Immunology 186, no. 1_Supplement (2011): 44.13. http://dx.doi.org/10.4049/jimmunol.186.supp.44.13.

Full text

Abstract:

Abstract Systemic juvenile idiopathic arthritis (SJIA) is a chronic autoinflammatory condition of childhood, characterized by remitting fever, transient rash, and relapsing arthritis. The association of macrophage activation syndrome with SJIA and clinical evidence implicating monocyte-derived cytokines IL-1 and IL-6 suggest a key role for monocyte in SJIA pathogenesis. We have previously found monocyte expansion during disease activity (flare), and a normal distribution of the monocyte subsets CD14hiCD16- and CD14loCD16+. We also observed elevated expression of monocyte surface markers CD14 a

APA, Harvard, Vancouver, ISO, and other styles

19

Minoia, Francesca, Francesca Bovis, Sergio Davì, et al. "Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis." Annals of the Rheumatic Diseases 78, no. 10 (2019): 1357–62. http://dx.doi.org/10.1136/annrheumdis-2019-215211.

Full text

Abstract:

ObjectiveTo develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA).MethodsThe clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20% constituted the validation sample. A Bayesian Model Averaging approach was used to assess the role of each clinical and laboratory v

APA, Harvard, Vancouver, ISO, and other styles

20

Alekseeva, Ekaterina I., Dariya D. Van’kova, Margarita A. Soloshenko, et al. "Pneumococcal Vaccine in Patients with Systemic Juvenile Idiopathic Arthritis Receiving Biologic Therapy: International Practice Review." Current Pediatrics 18, no. 2 (2019): 101–8. http://dx.doi.org/10.15690/vsp.v18i2.2012.

Full text

Abstract:

International practice of immunization against pneumococcus in patients with systemic juvenile idiopathic arthritis (SJIA) receiving biological therapy is generalized in this review. High efficiency and safety of pneumococcal vaccines in children with SJIA is presented. Numerous researches show the adequate immune response after vaccination as well as alongside with genetically engineered biologic drugs therapy. Prevention of pneumococcal disease in patients with SJIA reduces the risk of development of pneumococcal diseases severe complications.

APA, Harvard, Vancouver, ISO, and other styles

21

Petrongari, Duilio, Paola Di Filippo, Francesco Misticoni, et al. "Lung Involvement in Systemic Juvenile Idiopathic Arthritis: A Narrative Review." Diagnostics 12, no. 12 (2022): 3095. http://dx.doi.org/10.3390/diagnostics12123095.

Full text

Abstract:

Systemic juvenile idiopathic arthritis associated with lung disorders (sJIA-LD) is a subtype of sJIA characterized by the presence of chronic life-threatening pulmonary disorders, such as pulmonary hypertension, interstitial lung disease, pulmonary alveolar proteinosis and/or endogenous lipoid pneumonia, which were exceptionally rare before 2013. Clinically, these children show a striking dissociation between the relatively mild clinical manifestations (tachypnoea, clubbing and chronic cough) and the severity of the pulmonary inflammatory process. Our review describes sJIA-LD as having a repor

APA, Harvard, Vancouver, ISO, and other styles

22

Saper, Vivian E., Guangbo Chen, Gail H. Deutsch, et al. "Emergent high fatality lung disease in systemic juvenile arthritis." Annals of the Rheumatic Diseases 78, no. 12 (2019): 1722–31. http://dx.doi.org/10.1136/annrheumdis-2019-216040.

Full text

Abstract:

ObjectiveTo investigate the characteristics and risk factors of a novel parenchymal lung disease (LD), increasingly detected in systemic juvenile idiopathic arthritis (sJIA).MethodsIn a multicentre retrospective study, 61 cases were investigated using physician-reported clinical information and centralised analyses of radiological, pathological and genetic data.ResultsLD was associated with distinctive features, including acute erythematous clubbing and a high frequency of anaphylactic reactions to the interleukin (IL)-6 inhibitor, tocilizumab. Serum ferritin elevation and/or significant lymph

APA, Harvard, Vancouver, ISO, and other styles

23

Ailioaie, Laura Marinela, Constantin Ailioaie, and Gerhard Litscher. "Biomarkers in Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome and Their Importance in COVID Era." International Journal of Molecular Sciences 23, no. 21 (2022): 12757. http://dx.doi.org/10.3390/ijms232112757.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) and its complication, macrophage activation syndrome (sJIA-MAS), are rare but sometimes very serious or even critical diseases of childhood that can occasionally be characterized by nonspecific clinical signs and symptoms at onset—such as non-remitting high fever, headache, rash, or arthralgia—and are biologically accompanied by an increase in acute-phase reactants. For a correct positive diagnosis, it is necessary to rule out bacterial or viral infections, neoplasia, and other immune-mediated inflammatory diseases. Delays in diagnosis will result

APA, Harvard, Vancouver, ISO, and other styles

24

Kaleda, M., I. Nikishina, V. Matkava, A. Shapovalenko, E. Fedorov, and S. Salugina. "AB0725 MACROPHAGE ACTIVATION SYNDROME IN CHILDREN WITH RHEUMATIC DISEASES: ANALYSIS OF CASE SERIES." Annals of the Rheumatic Diseases 80, Suppl 1 (2021): 1393.3–1394. http://dx.doi.org/10.1136/annrheumdis-2021-eular.2098.

Full text

Abstract:

Background:Macrophage activation syndrome (MAS) is a rare, but severe life-threatening complication of chronic rheumatic disease (RD) in children, which associated with high risks of the multiple organ failure and mortality.Objectives:Tо analyze demographic, clinical and laboratory parameters, timing of MAS and disease outcome in patients (pts) with MAS and RD.Methods:The study included all pts of single center with RD, who developed the MAS. The diagnosis was recognized according to Classification criteria for MAS in sJIA [1].Results:We observed 52 pts with RD and MAS: 31 (59.6%) with sJIA, 1

APA, Harvard, Vancouver, ISO, and other styles

25

Alekseeva, E., R. Denisova, S. Valieva, et al. "P02-029 - CAPS or SJIA." Pediatric Rheumatology 11, Suppl 1 (2013): A136. http://dx.doi.org/10.1186/1546-0096-11-s1-a136.

Full text

APA, Harvard, Vancouver, ISO, and other styles

26

Kathmann, Wiebke. "Hoffnung für Patienten mit SJIA." pädiatrie hautnah 25, no. 2 (2013): 117. http://dx.doi.org/10.1007/s15014-013-0071-z.

Full text

APA, Harvard, Vancouver, ISO, and other styles

27

Kostik, Mikhail Mikhaylovich, Tatyana Serafimovna Likhacheva, Irina Aleksandrovna Chikova, et al. "Higher-dose canakinumab therapy for refractory macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: two case reports." Pediatrician (St. Petersburg) 5, no. 4 (2014): 14–19. http://dx.doi.org/10.17816/ped5414-19.

Full text

Abstract:

Macrophage activation syndrome (MAS) is a life-threatening, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) appears in non-remitted fever, cytopenia, coagulopathy, liver and CNS dysfunctions. Triggers of MAS could be disease activity, infections and medications. Known IL-1 is the key cytokine in pathogenesis of MAS and SJIA, and disease flare associated with increased amounts of different cytokines, especially IL-1β. Many cases of MAS are medically-refractory to traditional doses of cytokine inhibition and may require increased dosing of biologic cytokine blocka

APA, Harvard, Vancouver, ISO, and other styles

28

Lee, Pui Y., Grant S. Schulert, Scott W. Canna, et al. "Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis." Annals of the Rheumatic Diseases 79, no. 2 (2019): 225–31. http://dx.doi.org/10.1136/annrheumdis-2019-216030.

Full text

Abstract:

ObjectiveMacrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (sJIA) characterised by a vicious cycle of immune amplification that can culminate in overwhelming inflammation and multiorgan failure. The clinical features of MAS overlap with those of active sJIA, complicating early diagnosis and treatment. We evaluated adenosine deaminase 2 (ADA2), a protein of unknown function released principally by monocytes and macrophages, as a novel biomarker of MAS.MethodsWe established age-based normal ranges of peripheral blood ADA2 activity i

APA, Harvard, Vancouver, ISO, and other styles

29

Roth, Johannes. "Komplizierter Verlauf einer systemischen JIA." Arthritis und Rheuma 42, no. 02 (2022): 114–19. http://dx.doi.org/10.1055/a-1781-8225.

Full text

Abstract:

ZUSAMMENFASSUNGDie systemische juvenile idiopathische Arthritis (sJIA) kann sehr behandlungsresistent sein und es können erhebliche Komplikationen einschließlich einer interstitiellen Lungenerkrankung auftreten. Anhand der Fallgeschichte einer sJIA-Patientin mit fortgeschrittener Lungenerkrankung werden molekulardiagnostische Methoden, neue Behandlungsoptionen und ethische Überlegungen bei der Versorgung komplexer Patienten diskutiert.

APA, Harvard, Vancouver, ISO, and other styles

30

Canny, Susan, and Elizabeth Mellins. "New frontiers in the treatment of systemic juvenile idiopathic arthritis." F1000Research 6 (June 22, 2017): 971. http://dx.doi.org/10.12688/f1000research.11327.1.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) and its most significant complication, macrophage activation syndrome (MAS), have traditionally been treated with steroids and non-steroidal anti-inflammatory medications. However, the introduction of biologic medications that inhibit specific cytokines, such interleukins 1 and 6, has changed the treatment paradigm for sJIA patients. In this review, we discuss the therapies currently used in the treatment of sJIA as well as novel targets and approaches under consideration, including mesenchymal stromal cell therapy and JAK inhibitors. We also discu

APA, Harvard, Vancouver, ISO, and other styles

31

Krekhova, E., E. Alexeeva, T. Dvoryakovskaya, et al. "POS1312 DRUG SURVIVAL FOR IL-6 INHIBITOR TOCILIZUMAB: DATA FROM A SINGLE-CENTER OBSERVATION." Annals of the Rheumatic Diseases 80, Suppl 1 (2021): 938–39. http://dx.doi.org/10.1136/annrheumdis-2021-eular.2255.

Full text

Abstract:

Background:The efficacy of tocilizumab for treatment patients with systemic juvenile idiopathic arthritis (sJIA) was demonstrated before. We want to describe tocilizumab drug survival based on data from a single-center observation.Objectives:To analyze the drug survival of tocilizumab in patients with sJIA treated at the National Medical Research Center of Children`s health, Moscow, Russia.Methods:Medical records from sJIA patients treated with tocilizumab (TOC) were analyzed retrospectively from the National Medical Research Center of Children`s health, Moscow, Russia.Results:One hundred nine

APA, Harvard, Vancouver, ISO, and other styles

32

Alexeeva, Ekaterina I., Dariya D. Vankova, Tatyana M. Dvoryakovskaya, et al. "Efficacy of Pneumococcal Polysaccharide Conjugate Vaccine (13-valent, Adsorbed) in Patients with Systemic Juvenile Idiopathic Arthritis Treated with Genetically Engineered Biologic Drugs (Tocilizumab or Canakinumab): Prospective Cohort Study." Current Pediatrics 19, no. 3 (2020): 190–99. http://dx.doi.org/10.15690/vsp.v19i3.2114.

Full text

Abstract:

Background. Immunological potency of 13-valent pneumococcal vaccine (PCV-13) in children with systemic juvenile idiopathic arthritis (SJIA) is still unstudied. Estimates of the genetically engineered biologic drugs (GEBD) effects on pneumococcal vaccination results also remain controversial.Objective. The aim of the study was to explore the PCV-13 efficacy in patients with SJIA and who is on treatment with monoclonal antibodies against interleukin 6 receptor (tocilizumab) and interleukin 8 receptor beta (canakinumab).Methods. The study included patients under the age of 18 with SJIA in remissi

APA, Harvard, Vancouver, ISO, and other styles

33

Worley, Emily, Weijie Li, and Jordan T. Jones. "Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome." Case Reports in Pediatrics 2021 (August 11, 2021): 1–4. http://dx.doi.org/10.1155/2021/6567770.

Full text

Abstract:

Systemic juvenile idiopathic arthritis (sJIA) is a chronic, inflammatory disease of childhood, which is characterized by the combination of arthritis, serositis, daily, high-spiking fevers, and evanescent macular rash and can present with the life-threatening complication of macrophage activation syndrome (MAS). Children with Down syndrome (DS) have complex medical challenges related to abnormalities in their immune system, which can cause a broad spectrum of disease manifestations, which can occur atypically. Children with DS are at increased risk for arthritis and interstitial lung disease (

APA, Harvard, Vancouver, ISO, and other styles

34

Cepika, Alma-Martina, Romain Banchereau, Elodie Segura, et al. "A multidimensional blood stimulation assay reveals immune alterations underlying systemic juvenile idiopathic arthritis." Journal of Experimental Medicine 214, no. 11 (2017): 3449–66. http://dx.doi.org/10.1084/jem.20170412.

Full text

Abstract:

The etiology of sporadic human chronic inflammatory diseases remains mostly unknown. To fill this gap, we developed a strategy that simultaneously integrates blood leukocyte responses to innate stimuli at the transcriptional, cellular, and secreted protein levels. When applied to systemic juvenile idiopathic arthritis (sJIA), an autoinflammatory disease of unknown etiology, this approach identified gene sets associated with specific cytokine environments and activated leukocyte subsets. During disease remission and off treatment, sJIA patients displayed dysregulated responses to TLR4, TLR8, an

APA, Harvard, Vancouver, ISO, and other styles

35

Gorelik, Mark, Ndate Fall, Mekibib Altaye, et al. "Follistatin-like Protein 1 and the Ferritin/Erythrocyte Sedimentation Rate Ratio Are Potential Biomarkers for Dysregulated Gene Expression and Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis." Journal of Rheumatology 40, no. 7 (2013): 1191–99. http://dx.doi.org/10.3899/jrheum.121131.

Full text

Abstract:

Objective.Follistatin-like protein 1 (FSTL-1) is a secreted glycoprotein overexpressed in certain inflammatory diseases. Our objective was to correlate FSTL-1 levels with gene expression, known biomarkers, and measures of disease activity in systemic juvenile idiopathic arthritis (sJIA), including macrophage activation syndrome (MAS).Methods.FSTL-1 serum levels were measured by ELISA in 28 patients with sJIA, including 7 patients who developed MAS, and 30 healthy controls. Levels were correlated with erythrocyte sedimentation rate (ESR), ferritin, and soluble interleukin-2 receptor-α (sIL-2Rα)

APA, Harvard, Vancouver, ISO, and other styles

36

de Zegher, Francis, Nele Reynaert, Lien De Somer, Carine Wouters, and Mathieu Roelants. "Growth Failure in Children with Systemic Juvenile Idiopathic Arthritis and Prolonged Inflammation despite Treatment with Biologicals: Late Normalization of Height by Combined Hormonal Therapies." Hormone Research in Paediatrics 90, no. 5 (2018): 337–43. http://dx.doi.org/10.1159/000489778.

Full text

Abstract:

Background: Biologicals targeting the interleukin (IL)-1β or IL-6 pathway are becoming prime choices for the treatment of children with systemic juvenile idiopathic arthritis (sJIA). Up to 1 in 3 sJIA children receiving such treatment continues to have inflammatory activity and to require supra-physiological glucocorticoid doses which may reduce growth velocity for years and may lead to an extremely short stature for age, if not for life. Currently, there is no long-term proposal to normalize the adult height of these children with sJIA. Methods and Results: We present long-term (up to 10 year

APA, Harvard, Vancouver, ISO, and other styles

37

Yasin, Shima, Ndate Fall, Rachel A. Brown, et al. "IL-18 as a biomarker linking systemic juvenile idiopathic arthritis and macrophage activation syndrome." Rheumatology 59, no. 2 (2019): 361–66. http://dx.doi.org/10.1093/rheumatology/kez282.

Full text

Abstract:

Abstract Objectives Systemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with features of autoinflammation and high risk of macrophage activation syndrome (MAS). IL-18 has been shown to have key roles in sJIA and MAS. We aimed to examine IL-18 levels in sJIA in relation to disease activity and history of MAS and other disease biomarkers namely S100 proteins and CXCL9. Methods Total IL-18, CXCL9 and S100 proteins levels were determined in 40 sJIA patients, and IL-18 levels were compared between patients with regards to disease activity, history of MAS, and other biomarkers. Re

APA, Harvard, Vancouver, ISO, and other styles

38

Alexeeva, E., E. Krekhova, T. Dvoryakovskaya, et al. "THU0506 LONG-TERM EFFECTIVENESS AND SAFETY OF CANAKINUMAB AS A SECOND BIOLOGIC AFTER TOCILIZUMAB IN CHILDREN WITH EARLY AND LATE JIA WITH ACTIVE SYSTEMIC FEATURES." Annals of the Rheumatic Diseases 79, Suppl 1 (2020): 491.2–492. http://dx.doi.org/10.1136/annrheumdis-2020-eular.5706.

Full text

Abstract:

Background:Canakinumab (CAN) is often used as second biologics in juvenile idiopathic arthritis with active systemic features (sJIA). However, there are little information about its long-term efficacy and safety.Objectives:To evaluate the long-term effectiveness and safety of CAN as a second biologics after tocilizumab (TOC) in sJIA patients depending on the duration of the disease.Methods:Thirty-one patients were enrolled in this study: the group of early sJIA (with duration shorter than 2 years, 19 patients) and the group of late sJIA (with duration longer than 2 years, 12 patients). At the

APA, Harvard, Vancouver, ISO, and other styles

39

Pardeo, Manuela, Jianmei Wang, Nicolino Ruperto, et al. "Neutropenia During Tocilizumab Treatment Is Not Associated with Infection Risk in Systemic or Polyarticular-course Juvenile Idiopathic Arthritis." Journal of Rheumatology 46, no. 9 (2019): 1117–26. http://dx.doi.org/10.3899/jrheum.180795.

Full text

Abstract:

Objective.To determine whether neutropenia is associated with increased risk for infection in patients with systemic juvenile idiopathic arthritis (sJIA) and polyarticular-course juvenile idiopathic arthritis (pcJIA) treated with tocilizumab (TCZ).Methods.Data up to Week 104 from 2 phase III trials of intravenous TCZ in sJIA (n = 112;ClinicalTrials.gov,NCT00642460) and pcJIA (n = 188;ClinicalTrials.gov,NCT00988221) were pooled. Worst common toxicity criteria grade and lowest observed absolute neutrophil count (ANC) were identified for each patient. Associations between patient characteristics

APA, Harvard, Vancouver, ISO, and other styles

40

R, N., A. Jain, H. Muhammed, et al. "SAT0230 MACROPHAGE ACTIVATION SYNDROME IN SLE AND SYSTEMIC ONSET JIA: SIMILAR OR DISSIMILAR." Annals of the Rheumatic Diseases 79, Suppl 1 (2020): 1057.2–1058. http://dx.doi.org/10.1136/annrheumdis-2020-eular.4469.

Full text

Abstract:

Background:Macrophage activation syndrome (MAS) is a serious complication in rheumatic disease. Fever and hyperferritinemia are common in systemic onset JIA and cytopenias are common in SLE thus recognising MAS in them is a challenge.Objectives:We compared clinical, laboratory parameters, various classification criteria for MAS, and its outcome in SLE and sJIA.Methods:Clinical and laboratory data were extracted from clinician diagnosed cases of MAS with SLE/sJIA who were admitted between 2004-2018 at a tertiary care hospital. Percentage of patients satisfying Ravelli, International consensus,

APA, Harvard, Vancouver, ISO, and other styles

41

Batulevičiūtė, Lina, Violeta Radžiūnienė, Ramunė Vankevičienė, Odeta Kinčinienė, and Violeta Panavienė. "VAIKŲ NEAIŠKIOS KILMĖS KARŠČIAVIMAS." Medicinos teorija ir praktika 20, no. 4 (2014): 376–81. http://dx.doi.org/10.15591/mtp.2014.067.

Full text

Abstract:

Reikšminiai žodžiai: neaiškios kilmės karščiavimas, sisteminis jaunatvinis idiopatinis artritas, vaikai. Santrumpos: sJIA – sisteminis jaunatvinis idiopatinis artritas, MAS – makrofagų aktyvacijos sindromas, NKK – neaiškios kilmės karščiavimas. Neaiškios kilmės karščiavimas – karščiavimas ≥ 38,3 °C, trunkantis ilgiau nei 8 dienas, kai, įvertinus anamnezę, klinikinės apžiūros bei pradinių laboratorinių tyrimų duomenis, priežastis lieka nenustatyta. Pagrindinės karščiavimo priežastys vaikų amžiuje yra infekcijos, autoimuninės ligos ir navikai. Tarp reumatinių ligų dažniausias yra sisteminis jaun

APA, Harvard, Vancouver, ISO, and other styles

42

RUSSO, RICARDO A. G., and MARÍA M. KATSICAS. "Patients with Very Early-onset Systemic Juvenile Idiopathic Arthritis Exhibit More Inflammatory Features and a Worse Outcome." Journal of Rheumatology 40, no. 3 (2013): 329–34. http://dx.doi.org/10.3899/jrheum.120386.

Full text

Abstract:

Objective.Systemic juvenile idiopathic arthritis (SJIA) frequently leads to disability and damage. Predictive factors for a poor outcome include persistent systemic features and younger age at onset. We describe and analyze disease features in patients with early-onset (EO) SJIA (disease onset before age 18 mo) and compare them to patients with later-onset (LO) disease.Methods.Clinical features at onset, activity measures (occurrence of macrophage activation syndrome, remission), and outcome measures for disability [Childhood Health Assessment Questionnaire (CHAQ) ≥ 0.5] and damage [radiograph

APA, Harvard, Vancouver, ISO, and other styles

43

Bracaglia, Claudia, Kathy de Graaf, Denise Pires Marafon та ін. "Elevated circulating levels of interferon-γ and interferon-γ-induced chemokines characterise patients with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis". Annals of the Rheumatic Diseases 76, № 1 (2016): 166–72. http://dx.doi.org/10.1136/annrheumdis-2015-209020.

Full text

Abstract:

ObjectivesInterferon-γ (IFNγ) is the pivotal mediator in murine models of primary haemophagocytic lymphohistiocytosis (pHLH). Given the similarities between primary and secondary HLH (sec-HLH), including macrophage activation syndrome (MAS), we investigate the involvement of the IFNγ pathway in MAS by evaluating levels of IFNγ and of the induced chemokines, and their relation with laboratory parameters of MAS in systemic juvenile idiopathic arthritis (sJIA) patients with MAS and in a murine MAS model.MethodsThe Luminex multiplexing assay was used to assess serum levels of interleukin (IL)-1β,

APA, Harvard, Vancouver, ISO, and other styles

44

Zimmermann, Wolfgang. "Dauerhafte Symptomreduktion bei CAPS und sJIA." pädiatrie: Kinder- und Jugendmedizin hautnah 28, no. 1 (2016): 66. http://dx.doi.org/10.1007/s15014-016-0637-7.

Full text

APA, Harvard, Vancouver, ISO, and other styles

45

Onuora, Sarah. "Multi-pronged approach uncovers sJIA mechanisms." Nature Reviews Rheumatology 13, no. 11 (2017): 631. http://dx.doi.org/10.1038/nrrheum.2017.173.

Full text

APA, Harvard, Vancouver, ISO, and other styles

46

Kriulin, I. A., E. I. Alexeeva, I. Yu Shilkrot, and T. M. Dvoryakovskaya. "Secondary hemophagocytic lymphohistiocytosis: prognostic model and early markers in patients with systemic juvenile idiopathic arthritis. Results of a cohort retrospective study." Voprosy praktičeskoj pediatrii 17, no. 6 (2022): 17–24. http://dx.doi.org/10.20953/1817-7646-2022-6-17-24.

Full text

Abstract:

Background. Secondary hemophagocytic lymphohistiocytosis (sHLH) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) characterized by hyperinflammation and a variety of clinical and laboratory manifestations. This condition is also referred to as macrophage activation syndrome (MAS) in patients with rheumatic diseases, including those with sJIA. In this article, we use the term sHLH. Approximately 40% of sHLH cases are asymptomatic, especially in patients who receive biologicals. Thus, the development of a prognostic model and identification of early sHLH marker

APA, Harvard, Vancouver, ISO, and other styles

47

Kaleda, M. I., I. P. Nikishina, S. O. Salugina, E. S. Fedorov, and E. V. Nikolaeva. "MACROPHAGE ACTIVATION SYNDROME IN RHEUMATIC DISEASES IN CHILDREN: A RETROSPECTIVE STUDY." Pediatria. Journal named after G.N. Speransky 100, no. 5 (2021): 53–61. http://dx.doi.org/10.24110/0031-403x-2021-100-5-53-61.

Full text

Abstract:

Macrophage activation syndrome (MAS) is a rare life-threatening complication of rheumatic diseases (RD) that requires early recognition and adequate immediate treatment. Objective of the study: to identify the features of onset of RD in patients who developed MAS, the clinical and laboratory characteristics of the MAS, possible trigger factors and the timing of development. Materials and methods of research: 57 patients (20 boys and 37 girls) with RD who developed MAS were included in a retrospective continuous non-randomized study: 36 (63%) with systemic juvenile idiopathic arthritis (sJIA),

APA, Harvard, Vancouver, ISO, and other styles

48

Limenis, Elizaveta, Brian M. Feldman, Camille Achonu, et al. "Proposed Core Set of Items for Measuring Disease Activity in Systemic Juvenile Idiopathic Arthritis." Journal of Rheumatology 45, no. 1 (2017): 115–21. http://dx.doi.org/10.3899/jrheum.161534.

Full text

Abstract:

Objective.To date, there are no standardized disease activity tools for systemic juvenile idiopathic arthritis (sJIA). We developed a core set of disease activity measures for sJIA.Methods.We conducted a validation study in patients with sJIA recruited from 3 Canadian institutions. Disease activity scores were based on questionnaires, clinical factors, and laboratory measures. The physician’s global assessment was our criterion standard. We determined the strength of association of each item with the criterion standard. We then surveyed international experts to determine the top 10 items. Fina

APA, Harvard, Vancouver, ISO, and other styles

49

Tajkia, Gule, Syed Khairul Amin, Shamim Rima, Soma Halder, and Fabia Hannan. "Systemic Onset Juvenile Idiopathic Arthritis (sJIA) or Still's Disease: A Diagnostic Challenge; while Presented as Fever of Unknown Origin. 2 Cases." Anwer Khan Modern Medical College Journal 10, no. 1 (2019): 84–88. http://dx.doi.org/10.3329/akmmcj.v10i1.43667.

Full text

Abstract:

Systemic-onset juvenile idiopathic arthritis (sJIA) or Still's disease is a chronic inflammatory disease of unknown etiology belongs to the group of Juvenile Idiopathic Arthritis. In contrast to other JIA patients in whom the joint disease usually overshadows the more general symptomatology, in Systemic-onset juvenile idiopathic arthritis (sJIA) extra-articular features such as spiking fever, hepatosplenomegaly, lymphadenopathy, rash, pleurisy, or pericarditis, and vasculopathy are most prominent. Thus the onset of disease can be vary nonspecific and may suggest bacterial or viral infection, m

APA, Harvard, Vancouver, ISO, and other styles

50

Kirchner, M., B. Robinson, L. Strothmann, A. Sonnenschein, and W. Mannhardt-Laakmann. "In-vitro-Einfluss von Adalimumab und Anakinra auf das Zytokin-netzwerk bei Patienten mit oJIA und sJIA." Arthritis und Rheuma 35, no. 05 (2015): 323–30. http://dx.doi.org/10.1055/s-0037-1618388.

Full text

Abstract:

ZusammenfassungEinleitung: Die oJIA gilt als Autoimmun-erkrankung der adaptiven Immunität, die sJIA wird als Autoinflammationserkrankung gewertet. Bei beiden Verlaufsformen werden die proinflammatorischen Zytokine IL-1, IL-6 und TNF-α hochreguliert. Daher gilt die Inhibition proinflammatorischer Zytokine als geeignete therapeutische Strategie. Die Autoren untersuchten, welchen Einfluss die Blockade eines einzelnen Zytokins auf das Gleichgewicht des gesamten Zytokinsystems nimmt.Methoden: Hierzu wurde die Zytokinsekretion nach In-vitro-LPS-Stimulation und Hemmung von IL-1 und TNF-[uni03B1] bei

APA, Harvard, Vancouver, ISO, and other styles

We offer discounts on all premium plans for authors whose works are included in thematic literature selections. Contact us to get a unique promo code!