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Systemic juvenile idiopathic arthritis

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https://www.readbyqxmd.com/read/29345059/microrna-networks-associated-with-active-systemic-juvenile-idiopathic-arthritis-regulate-cd163-expression-and-anti-inflammatory-functions-in-macrophages-through-two-distinct-mechanisms
#1
Thuy Do, Rachel Tan, Mark Bennett, Mario Medvedovic, Alexei A Grom, Nan Shen, Sherry Thornton, Grant S Schulert
Systemic juvenile idiopathic arthritis (SJIA) is a severe childhood arthropathy with features of autoinflammation. Monocytes and macrophages in SJIA have a complex phenotype with both pro- and anti-inflammatory properties that combine features of several well characterized in vitro conditions used to activate macrophages. An important anti-inflammatory phenotype is expression of CD163, a scavenger receptor that sequesters toxic pro-inflammatory complexes that is highly expressed in both active SJIA and macrophage activation syndrome (MAS)...
January 2018: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#2
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29333911/the-challenge-of-pediatric-uveitis-tertiary-referral-center-experience-in-the-united-states
#3
Mariantonia Ferrara, Laura Eggenschwiler, Andrew Stephenson, Alyssa Montieth, Nakhoul Nakhoul, Rafael Araùjo-Miranda, C Stephen Foster
PURPOSE: To describe the distribution, clinical findings, visual outcomes, treatment, and complications of children with uveitis at a tertiary referral ophthalmic center. METHODS: Retrospective cohort study. We reviewed the medical records of all patients ≤16 years with uveitis referred to Massachusetts Eye Research and Surgery Institution from March 2005 to July 2016. RESULTS: Of 286 included children, 62.24% were female. Mean age of onset was 8...
January 15, 2018: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/29333701/the-childhood-arthritis-rheumatology-research-alliance-consensus-treatment-plans-towards-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#4
REVIEW
Sarah Ringold, Peter A Nigrovic, Brian M Feldman, George A Tomlinson, Emily von Scheven, Carol A Wallace, Adam M Huber, Laura E Schanberg, Suzanne C Li, Pamela F Weiss, Robert C Fuhlbrigge, Esi M Morgan, Yukiko Kimura
The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research approaches. Innovative comparative effectiveness approaches are needed to efficiently study treatment approaches and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes...
January 15, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29307035/juvenile-idiopathic-arthritis-magnetic-resonance-imaging-of-the-clinically-unaffected-knee
#5
E Charlotte van Gulik, Mendy M Welsink-Karssies, J Merlijn van den Berg, Dieneke Schonenberg-Meinema, Koert M Dolman, Anouk M Barendregt, Charlotte M Nusman, Mario Maas, Taco W Kuijpers, Robert Hemke
BACKGROUND: Synovial thickening detected on magnetic resonance imaging (MRI) is present in a significant number of children with clinically inactive juvenile idiopathic arthritis (JIA). OBJECTIVE: To evaluate patient characteristics and disease activity parameters in a cohort of children with clinically inactive JIA, both with and without synovial thickening, in order to clarify the observed discrepancy between clinical and MRI assessments. MATERIALS AND METHODS: We prospectively enrolled 52 clinically inactive JIA patients (median age 13...
January 6, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29306244/tnf-alpha-863c-a-promoter-and-tnfrii-196t-g-exonic-variationsmay-be-risk-factors-for-juvenile-idiopathic-arthritis
#6
Bahadır Batar, Sezen Özman, Kenan Barut, Özgür Kasapçopur, Mehmet Güven
Background/aim: Juvenile idiopathic arthritis (JIA) is a chronic complex autoimmune disease. Genetic and environmental factors increase the risk of JIA. It is accepted that alterations in immune system pathways play an important role in the pathogenesis of JIA. The aim of the study was to investigate the possible association between immune system regulatory gene polymorphisms and JIA in Turkish patients. Materials and methods: We analyzed eight polymorphisms, TNF-alpha-863 C > A, TNFRII 196 T > G, IL2-631 G > A, IL13-1112 C > T, CCR2 190 G > A, CCR5delta32, CTLA4-1661 A > G, and PTPN22 1858 C > T, in 76 patients with JIA and in 80 healthy controls, who were of a similar age and same sex...
December 19, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29304824/patient-characteristics-associated-with-response-to-nsaid-monotherapy-in-children-with-systemic-juvenile-idiopathic-arthritis
#7
Anjali Sura, Christopher Failing, Julie Sturza, Jasmine Stannard, Meredith Riebschleger
BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is an auto-inflammatory disease characterized by fever, arthritis, and ≥1 of rash, generalized lymphadenopathy, hepato/splenomegaly, and serositis. Non-steroidal anti-inflammatory drugs (NSAIDs) are among the initial treatments of sJIA, but there is currently no evidence indicating which children should undergo a trial of NSAID monotherapy and which should not. Our objective is to identify presentation characteristics which are associated with response and lack of response to a trial of NSAID monotherapy...
January 5, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29304243/hyperexcitability-of-the-central-nervous-system-in-children-with-chronic-pain-a-systematic-review
#8
Roselien Pas, Kelly Ickmans, Sophie Van Oosterwijck, Kelly Van der Cruyssen, Anthe Foubert, Laurence Leysen, Jo Nijs, Mira Meeus
Objective: Hyperexcitability of the central nervous system plays an important role in the development and maintenance of chronic pain in adults. This knowledge has led to improved treatment strategies within this population. In children, however, research on the presence of central hyperexcitability is scarce. To further investigate this topic in children with chronic pain, there is a need for a clear literature overview. Design: Systematic review. Methods: The literature search was performed using the electronic databases PubMed and Web of Science...
January 2, 2018: Pain Medicine: the Official Journal of the American Academy of Pain Medicine
https://www.readbyqxmd.com/read/29303703/reasons-for-inactive-disease-and-flare-in-systemic-onset-juvenile-idiopathic-arthritis-patients-during-tocilizumab-treatment
#9
Mikhail M Kostik, Eugenia A Isupova, Irina A Chikova, Margarita F Dubko, Vera V Masalova, Ludmila S Snegireva, Olga V Kalashnikova, Vyacheslav G Chasnyk
OBJECTIVES: The aim of our study was to evaluate disease courses and outcomes of sJIA children undergoing tocilizumab (TCZ) treatment, and to establish the predictors which distinguish inactive disease and disease flares. METHODS: Our retrospective study included 48 active sJIA children who were refractory to different anti-rheumatic drugs and who were then started on TCZ. The effectiveness of TCZ was assessed by the changes of sJIA attributed signs and symptoms and the remission was judged according to the Wallace (2004) criteria...
December 15, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29287303/high-levels-of-dek-autoantibodies-in-sera-of-polyarticular-jia-patients-and-in-early-flare-following-cessation-of-anti-tnf-therapy
#10
Nirit Mor-Vaknin, Miguel Rivas, Maureen Legendre, Smriti Mohan, Ye Yuanfan, Theresa Mau, Anne Johnson, Bin Huang, Lili Zhao, Yukiko Kimura, Steven J Spalding, Paula W Morris, Beth S Gottlieb, Karen Onel, Judyann C Olson, Barbara S Edelheit, Michael Shishov, Lawrence K Jung, Elaine A Cassidy, Sampath Prahalad, Murray H Passo, Timothy Beukelman, Jay Mehta, Edward H Giannini, Barbara S Adams, Daniel J Lovell, David M Markovitz
OBJECTIVE: The nuclear oncoprotein DEK is an autoantigen associated with juvenile idiopathic arthritis (JIA), especially the oligoarticular subtype. DEK is a secreted chemoatactic factor. Abundant DEK and DEK autoantibodies are found in the inflamed JIA synovium. In screening sera samples from two different JIA patient cohorts for DEK autoantibodies, we now further characterize the nature of DEK autoantibodies. METHODS: DEK autoantibody levels were analyzed in sera from 33 JIA patients, 13 patients with other inflammatory conditions, and 11 healthy controls and 89 sera samples from JIA patients undergoing anti-TNF therapy...
December 29, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29277097/systemic-review-agreement-between-the-latent-tuberculosis-screening-tests-among-patients-with-rheumatic-diseases
#11
Junhee Pyo, Soo-Kyung Cho, Dam Kim, Yoon-Kyoung Sung
Background/Aims: To estimate the level of agreement and positivity rates of latent tuberculosis infection (LTBI) tests prior to the use of tumor necrosis factor (TNF) inhibitors in relation to underlying rheumatic diseases and endemic tuberculosis levels. Methods: The Ovid-Medline, Embase, and Cochrane Libraries were searched for articles before October 2013 involving LTBI screening in rheumatic patients, including rheumatoid arthritis (RA), ankylosing spondylitis (AS), juvenile idiopathic arthritis (JIA), and psoriatic arthritis...
December 28, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/29247988/interleukin-18-biological-properties-and-role-in-disease-pathogenesis
#12
REVIEW
Gilles Kaplanski
Initially described as an interferon (IFN)γ-inducing factor, interleukin (IL)-18 is indeed involved in Th1 and NK cell activation, but also in Th2, IL-17-producing γδ T cells and macrophage activation. IL-18, a member of the IL-1 family, is similar to IL-1β for being processed by caspase 1 to an 18 kDa-biologically active mature form. IL-18 binds to its specific receptor (IL-18Rα, also known as IL-1R7) forming a low affinity ligand chain. This is followed by recruitment of the IL-18Rβ chain. IL-18 then uses the same signaling pathway as IL-1 to activate NF-kB and induce inflammatory mediators such as adhesion molecules, chemokines and Fas ligand...
January 2018: Immunological Reviews
https://www.readbyqxmd.com/read/29242269/siadh-in-systemic-jia-resolving-after-treatment-with-an-il-6-inhibitor
#13
Juanita K Hodax, Shara R Bialo, Ali Yalcindag
Interleukin-6 (IL-6) is implicated in the pathogenesis of both systemic juvenile idiopathic arthritis (SJIA) and syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but the 2 have not been previously described as occurring together. We report a case of a 6-year-old girl with symptoms of arthralgia, daily fevers, evanescent rash, lymphadenopathy, and laboratory evaluation showing elevated inflammatory markers, consistent with SJIA. At presentation, the patient had hyponatremia with a sodium level of 128 mEq/L...
December 14, 2017: Pediatrics
https://www.readbyqxmd.com/read/29240923/neutrophil-activation-signature-in-juvenile-idiopathic-arthritis-indicates-the-presence-of-low-density-granulocytes
#14
Kavitha Ramanathan, Anna Glaser, Hanna Lythgoe, Joanne Ong, Michael W Beresford, Angela Midgley, Helen L Wright
Objective: JIA is an autoimmune, inflammatory disease with involvement of innate and adaptive immune responses. However, the role of neutrophils in JIA pathogenesis remains unclear. This study aimed to identify and validate neutrophil gene expression signatures in JIA using public microarray datasets and new clinical samples. Methods: Three suitable datasets were analysed by significance analysis of microarray and Ingenuity. Neutrophils and peripheral blood mononuclear cells (PBMCs) were isolated from a new cohort of JIA patients and healthy paediatric controls (HCs)...
December 12, 2017: Rheumatology
https://www.readbyqxmd.com/read/29195002/validation-of-classification-criteria-of-macrophage-activation-syndrome-in-japanese-patients-with-systemic-juvenile-idiopathic-arthritis
#15
Masaki Shimizu, Mao Mizuta, Takahiro Yasumi, Naomi Iwata, Yuka Okura, Noriko Kinjo, Hiroaki Umebayashi, Tomohiro Kubota, Yasuo Nakagishi, Kenichi Nishimura, Masato Yashiro, Junko Yasumura, Kazuko Yamazaki, Hiroyuki Wakiguchi, Nami Okamoto, Masaaki Mori
OBJECTIVE: To validate whether the 2016 ACR/EULAR classification criteria of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA) is practical in the real world. METHODS: A combination of expert consensus and analysis of real patient data was conducted by a panel of 15 paediatric rheumatologists. Sixty five profiles comprised 18 patients with s-JIA-associated MAS and 47 patients with active s-JIA without evidence of MAS...
December 1, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29194108/epigenetics-of-inflammatory-arthritis
#16
Deepa Hammaker, Gary S Firestein
PURPOSE OF REVIEW: Aberrant epigenetic changes in DNA methylation, histone marks, and noncoding RNA expression regulate the pathogenesis of many rheumatic diseases. The present article will review the recent advances in the epigenetic profile of inflammatory arthritis and discuss diagnostic biomarkers and potential therapeutic targets. RECENT FINDINGS: Methylation signatures of fibroblast-like synoviocytes not only distinguish rheumatoid arthritis (RA) and osteoarthritis (OA), but also early RAs from late RAs or juvenile idiopathic arthritis...
November 30, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/29185965/ultrasound-changes-in-synovial-abnormalities-induced-by-treatment-in-juvenile-idiopathic-arthritis
#17
Stefano Lanni, E H Pieter van Dijkhuizen, Federica Vanoni, Stefania Viola, Francesca Magnaguagno, Gian Michele Magnano, Carlo Gandolfo, Angelo Ravelli, Clara Malattia
OBJECTIVES: To investigate the capacity of ultrasound (US) to detect improvement of synovial abnormalities induced by treatment in juvenile idiopathic arthritis (JIA). METHODS: Eighty-three joints (33 knees, 22 tibiotalar, 10 wrists, 9 elbows, 9 subtalar joints) of 33 patients with new-onset JIA were assessed by US at study entry and 6 months after a therapeutic intervention. Each joint was scored for grey-scale (GS) and power Doppler (PD) abnormalities according to a 4-point semiquantitative scale...
November 28, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29184458/update-on-the-management-of-systemic-juvenile-idiopathic-arthritis-and-role-of-il-1-and-il-6-inhibition
#18
REVIEW
Sriharsha Grevich, Susan Shenoi
Systemic juvenile idiopathic arthritis (SJIA) is a disease marked with arthritis and several features of systemic inflammation including fevers, rashes, hepatosplenomegaly, lymphadenopathy, and serositis. The presentation can be variable and arthritis can be a later feature. Macrophage activation syndrome can be a life-threatening complication of this illness and requires early recognition and prompt therapy. Advancements in understanding the biology of SJIA have led to the development of cytokine-targeted therapies, mainly interleukin-1 (IL-1) and IL-6 inhibitors that have significantly improved outcomes...
2017: Adolescent Health, Medicine and Therapeutics
https://www.readbyqxmd.com/read/29166924/experience-with-etanercept-tocilizumab-and-interleukin-1-inhibitors-in-systemic-onset-juvenile-idiopathic-arthritis-patients-from-the-biker-registry
#19
Gerd Horneff, Anna Carina Schulz, Jens Klotsche, Anton Hospach, Kirsten Minden, Ivan Foeldvari, Ralf Trauzeddel, Gerd Ganser, Frank Weller-Heinemann, Johannes Perter Haas
BACKGROUND: Treatment of systemic onset juvenile idiopathic arthritis JIA (sJIA), although dramatically improved, remains a challenge. Experience from clinical practice will be presented using data from the German Biologics register (BiKeR) for evaluation of efficacy and safety of treatment with etanercept (ETA), tocilizumab (TOC) and the interleukin-1 inhibitors anakinra and canakinumab (IL-1i) in sJIA. METHODS: Patients with sJIA documented in the BIKeR register, who were exposed to ETA, TOC or IL-1i were identified...
November 22, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29141313/-tocilizumab-for-refractory-systemic-juvenile-idiopathic-arthritis
#20
J M Lai, F Q Wu, Z X Zhou, M Kang, X L Huang, G X Su, S N Li, J Zhu, X N Wang
Objective: To evaluate the efficacy and side effects of tocilizumab for the treatment of systemic juvenile idiopathic arthritis. Method: In this prospective self case-control study, the children diagnosed with refractory systemic juvenile idiopathic arthritis admitted to Department of Rheumatism and Immunology of Children's Hospital Affiliated to Capital Institute of Pediatrics from December 2013 to June 2016 were enrolled and information before and after treatment of tocilizumab was analyzed. The tocilizumab was introvenously guttae in a dose of 8-12 mg/kg every 2 weeks...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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