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Systemic juvenile idiopathic arthritis

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https://www.readbyqxmd.com/read/29773271/juvenile-idiopathic-arthritis-associated-uveitis
#1
REVIEW
Ethan S Sen, A V Ramanan
Juvenile idiopathic arthritis (JIA) is the commonest rheumatic disease in children and JIA-associated uveitis its most frequent extra-articular manifestation. The uveitis is potentially sight-threatening and thus carries a considerable risk of morbidity with associated reduction in quality of life. The commonest form of uveitis seen in association with JIA is chronic anterior uveitis, which is almost always asymptomatic in the initial stages. Therefore, screening for JIA-associated uveitis in at-risk patients is essential...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773270/systemic-juvenile-idiopathic-arthritis-new-insights-into-pathogenesis-and-cytokine-directed-therapies
#2
REVIEW
Manuela Pardeo, Claudia Bracaglia, Fabrizio De Benedetti
Systemic juvenile idiopathic arthritis (sJIA) is considered as a polygenic autoinflammatory disease. The prominent systemic clinical features, the marked elevation of inflammatory markers, and the absence of autoantibodies make this disease very different from the other juvenile idiopathic arthritis (JIA) forms. Innate immune mechanisms appear to play a central role: the overproduction of inflammatory cytokines of innate immunity is a typical feature of sJIA. Increased understanding of the role of these cytokines has been translated into therapeutic approaches...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29766251/imaging-of-the-hip-in-juvenile-idiopathic-arthritis
#3
REVIEW
Susan Cheng Shelmerdine, Pier Luigi Di Paolo, Laura Tanturri de Horatio, Clara Malattia, Silvia Magni-Manzoni, Karen Rosendahl
Hip involvement is common and estimated to occur in approximately 35-63% of children with juvenile idiopathic arthritis (JIA). It is more prevalent in the aggressive systemic subtypes, with irreversible changes occurring as early as within 5 years of diagnosis. Whilst clinical parameters and joint examination can be useful for assessing disease severity, subclinical disease is known to exist and delayed treatment may herald a lifetime of disability and pain. Early recognition of JIA changes is therefore crucial in determining treatment options...
June 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29766250/juvenile-idiopathic-arthritis-the-role-of-imaging-from-a-rheumatologist-s-perspective
#4
REVIEW
Clara Malattia, Nikolay Tzaribachev, J Merlijn van den Berg, Silvia Magni-Manzoni
Alongside recent advances in treatment strategies for juvenile idiopathic arthritis (JIA), paediatric rheumatologists have taken increasing interest in the use of imaging. Magnetic resonance imaging (MRI) and musculoskeletal ultrasound, by providing more detailed information on disease activity than clinical examination and conventional radiography (CR), have become helpful diagnostic and managerial tools. The growing skeleton, however, with changing appearances over time, is still challenging in the establishment of valid scoring systems for pathological changes...
June 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29766248/imaging-of-the-knee-in-juvenile-idiopathic-arthritis
#5
REVIEW
Robert Hemke, Nikolay Tzaribachev, Anouk M Barendregt, J Merlijn van den Berg, Andrea S Doria, Mario Maas
In juvenile idiopathic arthritis (JIA), imaging is increasingly used in clinical practice. In this paper we discuss imaging of the knee, the clinically most commonly affected joint in JIA. In the last decade, a number of important steps have been made in the development of imaging outcome measures in children with JIA knee involvement. Ultrasound is undergoing a fast validation process, which should be accomplished within the next few years. The validation processes of MRI as an imaging biomarker for clinical trials in the JIA knee are at an advanced stage, with important data available on the feasibility, reliability and validity of the Juvenile Arthritis MRI Scoring system...
June 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29761223/serum-profile-of-transferrin-isoforms-in-juvenile-idiopathic-arthritis-a-preliminary-study
#6
Ewa Gruszewska, Magdalena Sienkiewicz, Paweł Abramowicz, Jerzy Konstantynowicz, Monika Gudowska-Sawczuk, Lech Chrostek, Bogdan Cylwik
It is reported that alterations in protein glycosylation are present in adult rheumatic diseases; however, the data related to pediatric rheumatic conditions are very scarce. The aim of this study was to assess the effect of juvenile idiopathic arthritis (JIA) on the serum glycosylation profile of transferrin isoforms. Twenty-five patients with different clinical forms of an active JIA and 22 healthy controls were studied. Serum samples were analyzed by capillary electrophoresis on MINICAP electrophoretic system (Sebia, France) to determine the levels of transferrin isoforms...
May 14, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29717335/a-novel-radiographic-scoring-system-for-growth-abnormalities-and-structural-change-in-children-with-juvenile-idiopathic-arthritis-of-the-hip
#7
Susan C Shelmerdine, Pier Luigi Di Paolo, Jasper F M M Rieter, Clara Malattia, Laura Tanturri de Horatio, Karen Rosendahl
BACKGROUND: Approximately 20-50% of children with juvenile idiopathic arthritis (JIA) have hip involvement within 6 years of diagnosis. Scoring systems for hip-related radiographic changes are lacking. OBJECTIVE: To examine precision of potential radiographic variables and to suggest a scoring system. MATERIALS AND METHODS: We reviewed a set of 75 pelvic radiographs from 75 children with JIA hip involvement across two European centres. We assessed findings of (1) destructive change and (2) growth abnormality, according to a pre-defined scoring system...
May 1, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29707409/juvenile-idiopathic-arthritis-associated-with-combined-jp-hht-syndrome-a-novel-phenotype-associated-with-a-novel-variant-in-smad4
#8
Juliet Chhay Bishop, Jacquelyn Francis Britton, Anne M Murphy, Sangeeta Sule, Sally Mitchell, Clifford Takemoto, Joseph M Collaco, Wikrom Karnsakul, Carmelo Cuffari, Edith Dietz, Joann Bodurtha
Juvenile polyposis (JP) syndrome is characterized by multiple hamartomatous polyps of the gastrointestinal tract. Hereditary hemorrhagic telangiectasia (HHT) is a vascular dysplasia characterized by telangiectasia in the skin, mucous membranes, and arteriovenous malformations in other organs. Individuals with JP-HHT syndrome have variable features of both rare disorders, attributed to heterozygous mutations in the SMAD4 gene. Systemic juvenile idiopathic arthritis (JIA) is a severe, chronic disease marked by arthritis and systemic inflammation for which the cause remains unknown...
June 2018: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/29685542/normal-mri-findings-of-the-knee-in-patients-with-clinically-active-juvenile-idiopathic-arthritis
#9
E Charlotte van Gulik, Robert Hemke, Mendy M Welsink-Karssies, Dieneke Schonenberg-Meinema, Koert M Dolman, Anouk M Barendregt, Charlotte M Nusman, Mario Maas, Taco W Kuijpers, J Merlijn van den Berg
OBJECTIVE: In a number of patients with clinically active juvenile idiopathic arthritis (JIA), contrast-enhanced MRI shows no signs of synovitis. The objective of this study was to assess the frequency and the patient characteristics in clinically active JIA patients in which MRI showed no signs of synovitis. METHODS: From our cohort of 313 patients in which contrast-enhanced MRI of the knee had been performed, we selected 72 JIA patients with clinically active disease involving the target joint...
May 2018: European Journal of Radiology
https://www.readbyqxmd.com/read/29683837/role-of-interleukin-37-as-a-novel-proangiogenic-factor-in-juvenile-idiopathic-arthritis
#10
Amal Mohamad El-Barbary, Manal Shawky Hussein, Samah Hamdy Almedany, Elsayed Mohamad Rageh, Alaa Mohamed Alsalawy, Marwa Ahmed Aboelhawa, Radwa Mostafa Elkholy, Noha Mohamed Shafik, Ahmed Shukry Elharoun
OBJECTIVE: The aim of this study was to investigate interleukin 37 (IL-37) levels in the serum and synovial fluid of patients with juvenile idiopathic arthritis (JIA), its expression in peripheral blood mononuclear cells, and correlation with disease activity and angiogenesis. METHODS: Seventy JIA patients and 50 control subjects were examined. The Juvenile Arthritis Disease Activity Score in 27 joints (JADAS-27) was calculated. Immunoassays were used to measure the serum and synovial fluid levels of IL-37, vascular endothelial growth factor (VEGF), soluble VEGF receptor 1 (sVEGF-R1), and sVEGF-R2...
April 16, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29672804/imaging-features-of-the-juvenile-inflammatory-arthropathies
#11
Iwona Sudoł-Szopińska, Lennart Jans, Anne Grethe Jurik, Robert Hemke, Iris Eshed, Nathalie Boutry
We discuss the imaging of several juvenile inflammatory arthropathies including juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis, and chronic recurrent multifocal osteomyelitis. Juvenile idiopathic arthritis is the most common autoimmune chronic systemic disease of connective tissue in children. The remaining systemic juvenile connective tissue diseases are rare. However, they require early diagnosis and initiation of treatment to prevent injury, not only to the musculoskeletal system but also to the internal organs, and even death...
April 2018: Seminars in Musculoskeletal Radiology
https://www.readbyqxmd.com/read/29663156/anakinra-treatment-in-macrophage-activation-syndrome-a-single-center-experience-and-systemic-review-of-literature
#12
Hafize Emine Sönmez, Selcan Demir, Yelda Bilginer, Seza Özen
Our aim was to report our experiences of pediatric macrophage activation syndrome (MAS) patients treated with anakinra and to review previous studies reporting anakinra treatment in pediatric MAS patients associated with systemic juvenile idiopathic arthritis (sJIA) or autoinflammatory diseases (AIDs). The study group consisted of pediatric MAS patients due to sJIA or AIDs, followed up in the Pediatric Rheumatology Unit of Hacettepe University between January 2015 and January 2017 and treated with anakinra (anti-IL1)...
April 16, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29662164/transcription-factors-operate-across-disease-loci-with-ebna2-implicated-in-autoimmunity
#13
John B Harley, Xiaoting Chen, Mario Pujato, Daniel Miller, Avery Maddox, Carmy Forney, Albert F Magnusen, Arthur Lynch, Kashish Chetal, Masashi Yukawa, Artem Barski, Nathan Salomonis, Kenneth M Kaufman, Leah C Kottyan, Matthew T Weirauch
Explaining the genetics of many diseases is challenging because most associations localize to incompletely characterized regulatory regions. Using new computational methods, we show that transcription factors (TFs) occupy multiple loci associated with individual complex genetic disorders. Application to 213 phenotypes and 1,544 TF binding datasets identified 2,264 relationships between hundreds of TFs and 94 phenotypes, including androgen receptor in prostate cancer and GATA3 in breast cancer. Strikingly, nearly half of systemic lupus erythematosus risk loci are occupied by the Epstein-Barr virus EBNA2 protein and many coclustering human TFs, showing gene-environment interaction...
April 16, 2018: Nature Genetics
https://www.readbyqxmd.com/read/29652654/patients-with-juvenile-idiopathic-arthritis-become-adults-the-role-of-transitional-care
#14
REVIEW
Fabrizio Conti, Irene Pontikaki, Mariella D'Andrea, Angelo Ravelli, Fabrizio De Benedetti
Most juvenile idiopathic arthritis (JIA) patients need to attend adult rheumatology centres to continue the clinical management of their disease and to receive adequate long-term treatment. Transition from the paediatric to the adult health care team is a critical moment in the clinical history of these patients, but unfortunately, about 50% of the transfer processes to adult rheumatology are not successful, putting these patients at high risk of unfavourable outcomes. There are several obstacles to the success of transitional care for JIA patients, such as the absence of specific criteria for the assessment of disease activity, the lack of specific treatment recommendations for JIA adult patients, the poor adolescent-specific training for adult rheumatologists, and the shortage of resources...
April 13, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29651907/evidence-based-clinical-practice-guideline-for-adult-still-s-disease
#15
Toshihide Mimura, Yuya Kondo, Akihide Ohta, Masahiro Iwamoto, Akiko Ota, Nami Okamoto, Yasushi Kawaguchi, Hajime Kono, Yoshinari Takasaki, Shuji Takei, Norihiro Nishimoto, Manabu Fujimoto, Yu Funakubo Asanuma, Akio Mimori, Naoko Okiyama, Shunta Kaneko, Hiroyuki Takahashi, Masahiro Yokosawa, Takayuki Sumida
OBJECTIVES: Using an expert- and data-driven methodology, we have constructed the first clinical practice guidelines (CPGs) for adult Still's disease (ASD) after complete systematic review (SR) of the literature based upon the Medical Information Network Distribution Service (Minds) procedure. METHODS: The CPG committee for ASD organized by the Research Team for Autoimmune Diseases, the Research Program for Intractable Disease of the Japanese Ministry of Health, Labour, and Welfare has developed CPG for ASD 2017, according to the procedure proposed by Minds...
May 9, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29643108/treating-juvenile-idiopathic-arthritis-to-target-recommendations-of-an-international-task-force
#16
Angelo Ravelli, Alessandro Consolaro, Gerd Horneff, Ronald M Laxer, Daniel J Lovell, Nico M Wulffraat, Jonathan D Akikusa, Sulaiman M Al-Mayouf, Jordi Antón, Tadej Avcin, Roberta A Berard, Michael W Beresford, Ruben Burgos-Vargas, Rolando Cimaz, Fabrizio De Benedetti, Erkan Demirkaya, Dirk Foell, Yasuhiko Itoh, Pekka Lahdenne, Esi M Morgan, Pierre Quartier, Nicolino Ruperto, Ricardo Russo, Claudia Saad-Magalhães, Sujata Sawhney, Christiaan Scott, Susan Shenoi, Joost F Swart, Yosef Uziel, Sebastiaan J Vastert, Josef S Smolen
Recent therapeutic advances in juvenile idiopathic arthritis (JIA) have made remission an achievable goal for most patients. Reaching this target leads to improved outcomes. The objective was to develop recommendations for treating JIA to target. A Steering Committee formulated a set of recommendations based on evidence derived from a systematic literature review. These were subsequently discussed, amended and voted on by an international Task Force of 30 paediatric rheumatologists in a consensus-based, Delphi-like procedure...
June 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29622022/il-6-blockade-in-systemic-juvenile-idiopathic-arthritis-achievement-of-inactive-disease-and-remission-data-from-the-german-aid-registry
#17
M Bielak, E Husmann, N Weyandt, J-P Haas, B Hügle, G Horneff, U Neudorf, T Lutz, E Lilienthal, T Kallinich, K Tenbrock, R Berendes, T Niehues, H Wittkowski, E Weißbarth-Riedel, G Heubner, P Oommen, J Klotsche, Dirk Foell, E Lainka
BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in the pathogenesis has been ascribed to proinflammatory cytokines like interleukin-6 (IL-6), and effective drugs inhibiting their signaling are being developed. This study evaluates sJIA patients treated with the IL-6 inhibitor tocilizumab (TCZ) concerning clinical response rate, disease course and adverse effects in a real-life clinical setting...
April 5, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29618462/allogeneic-hematopoietic-stem-cell-transplantation-for-severe-refractory-juvenile-idiopathic-arthritis
#18
Juliana M F Silva, Fani Ladomenou, Ben Carpenter, Sharat Chandra, Petr Sedlacek, Renata Formankova, Vicky Grandage, Mark Friswell, Andrew J Cant, Zohreh Nademi, Mary A Slatter, Andrew R Gennery, Sophie Hambleton, Terence J Flood, Giovanna Lucchini, Robert Chiesa, Kanchan Rao, Persis J Amrolia, Paul Brogan, Lucy R Wedderburn, Julie M Glanville, Rachael Hough, Rebecca Marsh, Mario Abinun, Paul Veys
Patients with juvenile idiopathic arthritis (JIA) can experience a severe disease course, with progressive destructive polyarthritis refractory to conventional therapy with disease-modifying antirheumatic drugs including biologics, as well as life-threatening complications including macrophage activation syndrome (MAS). Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a potentially curative immunomodulatory strategy for patients with such refractory disease. We treated 16 patients in 5 transplant centers between 2007 and 2016: 11 children with systemic JIA and 5 with rheumatoid factor-negative polyarticular JIA; all were either refractory to standard therapy, had developed secondary hemophagocytic lymphohistiocytosis/MAS poorly responsive to treatment, or had failed autologous HSCT...
April 10, 2018: Blood Advances
https://www.readbyqxmd.com/read/29614573/-eperythrozoonosis-complicated-with-hemophagocytic-syndrome-report-of-four-cases-and-review-of-literature
#19
J G Li, D Zhang, Z X Zhou, S N Li, M Kang, J M Lai
Objective: To analyze the clinical characteristics of eperythrozoonosis complicated with hemophagocytic syndrome (HPS) in 4 children. Methods: Four patients diagnosed with eperythrozoonosis complicated with HPS in the Children's Hospital Affiliated Capital Institute of Pediatrics during the period from June 2014 to July 2016 were enrolled. The clinical manifestations, laboratory examination data and therapeutic strategies were analyzed. A literature search (search terms included 'eperythrozoonosis' and 'hemophagocytic syndrome') was conducted using CNKI, Wanfang database, Chinese biomedical literature database and PubMed to include recently published studies (searched from the database establishment to January 2017)...
April 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29609200/il1rn-variation-influences-both-disease-susceptibility-and-response-to-human-recombinant-il-1ra-therapy-in-systemic-juvenile-idiopathic-arthritis
#20
Victoria L Arthur, Emily Shuldiner, Elaine F Remmers, Anne Hinks, Alexei A Grom, Dirk Foell, Alberto Martini, Marco Gattorno, Seza Özen, Sampath Prahalad, Andrew S Zeft, John F Bohnsack, Norman T Ilowite, Elizabeth D Mellins, Ricardo Russo, Claudio Len, Sheila Oliveira, Rae S M Yeung, Alan M Rosenberg, Lucy R Wedderburn, Jordi Anton, Johannes-Peter Haas, Angela Rösen-Wolff, Kirsten Minden, Ann Marie Szymanski, Wendy Thomson, Daniel L Kastner, Patricia Woo, Michael J Ombrello
OBJECTIVE: To determine whether systemic juvenile idiopathic arthritis (sJIA) susceptibility loci identified by candidate gene studies demonstrated association with sJIA in the largest study population assembled to date. METHODS: Single nucleotide polymorphisms (SNPs) from 11 previously reported sJIA risk loci were examined for association in 9 populations, including 770 sJIA cases and 6947 control subjects. The effect of sJIA-associated SNPs on gene expression was evaluated in silico in paired whole genome and RNA sequencing data from lymphoblastoid cell lines (LCL) of 373 European 1000 Genomes Project subjects...
April 2, 2018: Arthritis & Rheumatology
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