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Systemic juvenile idiopathic arthritis

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https://www.readbyqxmd.com/read/28202118/-clinical-and-laboratory-features-of-macrophage-activation-syndrome
#1
Li Guo, Mei-Ping Lu, Gui-Juan Dong, Li-Ping Teng, Yi-Ping Xu, Li-Xia Zou, Qi Zheng
OBJECTIVE: To study the clinical and laboratory features of macrophage activation syndrome (MAS) at the early stage of diagnosis, and to explore a method for early identification of MAS. METHODS: A retrospective analysis was performed for the demographic data, clinical and laboratory features, and treatment outcomes of 21 MAS patients. RESULTS: Of the 21 MAS patients, 14 had systemic juvenile idiopathic arthritis, 5 had Kawasaki disease (KD), and 2 had connective tissue disease (CTD) as primary diseases...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28182665/padi4-and-the-hla-drb1-shared-epitope-in-juvenile-idiopathic-arthritis
#2
Kaori Hisa, Masakatsu D Yanagimachi, Takuya Naruto, Takako Miyamae, Masako Kikuchi, Rhoki Hara, Tomoyuki Imagawa, Shumpei Yokota, Masaaki Mori
OBJECTIVE: Both genetic and environmental factors are associated with susceptibility to juvenile idiopathic arthritis (JIA). Many studies have reported that both a 'shared epitope' (SE) encoded by several HLA-DRB1 alleles and the peptidyl arginine deiminase type 4 (PADI4) gene polymorphisms are associated with susceptibility to rheumatoid arthritis (RA). However, it is uncertain whether JIA and RA share the latter genetic risk factor. Therefore, here we investigated relationships between HLA-SE and PADI4 polymorphisms with clinical subtypes of JIA...
2017: PloS One
https://www.readbyqxmd.com/read/28178439/juvenile-idiopathic-arthritis-in-the-era-of-international-cooperation
#3
REVIEW
Yosef Uziel
Juvenile idiopathic arthritis (JIA) is the most common chronic disease of childhood. Improved understanding of its pathogenesis has led to international cooperation in clinical studies. Multicenter, international collaborations and research facilitate rapid enrollment of enough patients to enable a variety of studies, including those of epidemiology, diagnostic and classification criteria, genetic disease predisposition, pathogenesis, outcomes, and treatment protocols. In the last 20 years, the vision of the Pediatric Rheumatology International Trial Organization (PRINTO) has become a reality of worldwide collaboration in pediatric rheumatology research, including North American and European research groups...
January 30, 2017: Rambam Maimonides Medical Journal
https://www.readbyqxmd.com/read/28176230/inflammatory-markers-and-disease-activity-in-juvenile-idiopathic-arthritis
#4
Sumantra Sarkar, Md Mahboob Alam, Gargi Das, Supratim Datta
OBJECTIVE: To evaluate the post treatment changes in disease activity and inflammatory markers over time in longitudinal follow-up involving different subtypes of juvenile idiopathic arthritis (JIA) patients. METHODS: This prospective longitudinal study, carried out over a period of 2 y, included JIA patients, both old and new, with high disease activity. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ferritin, CHAQ (Childhood Health Assessment Questionnaire) score and JADAS27 (Juvenile Arthritis Disease Activity score with 27 active joint counts) were estimated at the initial visit, 6 mo, 12 mo and 18 mo of follow-up...
February 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28166785/a-comparison-of-three-treatment-strategies-in-recent-onset-non-systemic-juvenile-idiopathic-arthritis-initial-3-months-results-of-the-best-for-kids-study
#5
P C E Hissink Muller, D M C Brinkman, D Schonenberg, Y Koopman-Keemink, I C J Brederije, W P Bekkering, T W Kuijpers, M A J van Rossum, L W A van Suijlekom-Smit, J M van den Berg, C F Allaart, R Ten Cate
BACKGROUND: Combination therapy with prednisone or etanercept may induce earlier and/or more improvement in disease activity in Disease Modifying Anti Rheumatic Drug (DMARD) naïve non-systemic Juvenile Idiopathic Arthritis (JIA) patients. Here we present three months clinical outcome of initial treatments of the BeSt-for-Kids study. METHODS: Included patients were randomized to either: 1. initial DMARD-monotherapy (sulfasalazine (SSZ) or methotrexate (MTX)), 2...
February 6, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28165841/survey-of-attitudes-of-non-pediatric-rheumatologists-among-councilors-of-the-japan-college-of-rheumatology-regarding-transitional-care
#6
Takako Miyamae, Syuji Takei, Yasuhiko Itoh, Hisashi Yamanaka
Objectives The transition from pediatric to adult healthcare systems has recently received worldwide attention. Surveys of the attitudes of Japanese non-pediatric rheumatologists regarding transitional care were conducted. Methods Non-pediatric rheumatologists among councilors of the Japan College of Rheumatology were enrolled in the surveys. Experiences of adult patients with childhood-onset rheumatic diseases, ideal medical care for these patients, and factors that made the transition to adult care difficult were examined via e-mail...
February 6, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28162159/-the-value-of-different-antibodies-detection-in-diagnosis-of-rheumatism-with-uveitis
#7
X F Xu, J Zhang, L Cui, Y H Wang, Y Yue, L Chi, J Bai, H M Li, X X Lu
Objective: To investigate the value of HLA-B27 antigen, antinuclear antibody (ANA), anti-dsDNA and Anti-Neutrophil Cytoplasmic Antibodies (ANCA) detection in diagnosis of rheumatism with uveitis. Methods: Seven hundred and twenty four newly diagnosed patients with non-infection uveitis in Beijing Tongren Hospital from March 2012 to March 2016 who long-term lived in Beijing and its surrounding areas were continuously enrolled. HLA-B27 antigen expressions in peripheral blood lymphocytes and ANA, anti-dsDNA and ANCA levels in serum were tested...
January 24, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28161232/-correctly-address-the-cause-of-hemophagocytic-lymphohistiocytosis
#8
M Penel-Page, B Ben Said, A Phan, L Hees, C Hartmann-Merlin, S Girard, Y Gillet, A Belot
Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe syndrome usually associated with a cytotoxicity deficiency, which leads to an excess of immune response driven by activated macrophages and cytotoxic T cells. In children, HLH can be genetic, as part of a familial lymphohistiocytosis, or secondary: the most frequent causes are systemic-onset juvenile idiopathic arthritis, hematological malignancies, and severe infections, especially with Ebstein-Barr virus or leishmaniosis. We report on the case of a 3-year-old girl with no past medical history, who presented inaugural Pseudomonas aeruginosa maxillary osteitis, with secondary HLH...
February 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28153169/juvenile-idiopatic-arthritis-in-orthodontics-case-report-with-a-6-year-follow-up
#9
Luciane Macedo de Menezes, Susana Maria Deon Rizzatto, Eduardo Martinelli Santayana de Lima, Paulo Ricardo Baccarin Matje, Maria Mercedes Picarelli
Juvenile idiopathic arthritis is a childhood disease that causes joint swelling and pain, and limitation in the range of joint movements. It is characterized by progressive destruction of the joints. Juvenile idiopathic arthritis is the most frequent systemic inflammatory disease of the temporomandibular joint. When the temporomandibular joint is involved, orthodontic treatment becomes more challenging. This case report shows the treatment of a young patient with a Class II subdivision malocclusion and juvenile idiopathic arthritis...
February 2017: American Journal of Orthodontics and Dentofacial Orthopedics
https://www.readbyqxmd.com/read/28148700/the-omeract-ultrasound-group-a-report-from-the-omeract-2016-meeting-and-perspectives
#10
Lene Terslev, Annamaria Iagnocco, George A W Bruyn, Esperanza Naredo, Jelena Vojinovic, Paz Collado, Nemanja Damjanov, Andrew Filer, Georgios Filippou, Stephanie Finzel, Frederique Gandjbakhch, Kei Ikeda, Helen I Keen, Marion C Kortekaas, Silvia Magni-Manzoni, Sarah Ohrndorf, Carlos Pineda, Viviana Ravagnani, Bethan Richards, Ilfita Sahbudin, Wolfgang A Schmidt, Heidi J Siddle, Maria S Stoenoiu, Marcin Szkudlarek, Nikolay Tzaribachev, Maria-Antonietta D'Agostino
OBJECTIVE: To provide an update from the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group on the progress for defining ultrasound (US) minimal disease activity threshold at joint level in rheumatoid arthritis (RA) and for standardization of US application in juvenile idiopathic arthritis (JIA). METHODS: For minimal disease activity, healthy controls (HC) and patients with early arthritis (EA) who were naive to disease-modifying antirheumatic drugs were recruited from 2 centers...
February 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28143550/spectrum-of-paediatric-rheumatic-diseases-in-nigeria
#11
Babatunde Hakeem Olaosebikan, Olufemi Oladipo Adelowo, Barakat Adeola Animashaun, Richard Oluyinka Akintayo
BACKGROUND: Paediatric rheumatology service in Sub-Sahara African is virtually not available as there is a shortage of paediatric rheumatologists and other rheumatology health professionals. We aim to describe the clinical spectrum and the frequencies of paediatric rheumatic diseases (PRDs) in Lagos State University Teaching Hospital (LASUTH), Lagos, Nigeria. METHODS: This is a retrospective review of patients with PRDs seen over a five year period (March 2010 to February 2016) at the rheumatology clinic and children ward of LASUTH...
January 31, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28137405/contraception-for-adolescents-with-chronic-rheumatic-diseases
#12
Benito Lourenço, Katia T Kozu, Gabriela N Leal, Marco F Silva, Elisabeth G C Fernandes, Camila M P França, Fernando H C Souza, Clovis A Silva
Contraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE), antiphospholipid syndrome (APS), juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM). Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Combined oral contraceptives (COC) are strictly prohibited for JSLE and APS patients with positive antiphospholipid antibodies...
January 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28129106/a-synthetic-mammalian-therapeutic-gene-circuit-for-sensing-and-suppressing-inflammation
#13
Anže Smole, Duško Lainšček, Urban Bezeljak, Simon Horvat, Roman Jerala
Inflammation, which is a highly regulated host response against danger signals, may be harmful if it is excessive and deregulated. Ideally, anti-inflammatory therapy should autonomously commence as soon as possible after the onset of inflammation, should be controllable by a physician, and should not systemically block beneficial immune response in the long term. We describe a genetically encoded anti-inflammatory mammalian cell device based on a modular engineered genetic circuit comprising a sensor, an amplifier, a "thresholder" to restrict activation of a positive-feedback loop, a combination of advanced clinically used biopharmaceutical proteins, and orthogonal regulatory elements that linked modules into the functional device...
January 4, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28115015/early-changes-in-gene-expression-and-inflammatory-proteins-in-systemic-juvenile-idiopathic-arthritis-patients-on-canakinumab-therapy
#14
Arndt H Brachat, Alexei A Grom, Nico Wulffraat, Hermine I Brunner, Pierre Quartier, Riva Brik, Liza McCann, Huri Ozdogan, Lidia Rutkowska-Sak, Rayfel Schneider, Valeria Gerloni, Liora Harel, Maria Terreri, Kristin Houghton, Rik Joos, Daniel Kingsbury, Jorge M Lopez-Benitez, Stephan Bek, Martin Schumacher, Marie-Anne Valentin, Hermann Gram, Ken Abrams, Alberto Martini, Daniel J Lovell, Nanguneri R Nirmala, Nicolino Ruperto
BACKGROUND: Canakinumab is a human anti-interleukin-1β (IL-1β) monoclonal antibody neutralizing IL-1β-mediated pathways. We sought to characterize the molecular response to canakinumab and evaluate potential markers of response using samples from two pivotal trials in systemic juvenile idiopathic arthritis (SJIA). METHODS: Gene expression was measured in patients with febrile SJIA and in matched healthy controls by Affymetrix DNA microarrays. Transcriptional response was assessed by gene expression changes from baseline to day 3 using adapted JIA American College of Rheumatology (aACR) response criteria (50 aACR JIA)...
January 23, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28095869/macrophage-activation-syndrome-different-mechanisms-leading-to-a-one-clinical-syndrome
#15
REVIEW
Claudia Bracaglia, Giusi Prencipe, Fabrizio De Benedetti
BACKGROUND: Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages. MAIN CONTENT: MAS is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH). The reason is that MAS shares clinical and laboratory features with primary genetic HLH (pHLH)...
January 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28094589/leucine-rich-%C3%AE-2-glycoprotein-as-the-acute-phase-reactant-to-detect-systemic-juvenile-idiopathic-arthritis-disease-activity-during-anti-interleukin-6-blockade-therapy-a-case-series
#16
Masaki Shimizu, Yasuo Nakagishi, Natsumi Inoue, Mao Mizuta, Akihiro Yachie
OBJECTIVES: To assess the role of leucine-rich α2-glycoprotein (LRG) as a biomarker for monitoring systemic juvenile idiopathic arthritis (s-JIA) disease activity during interleukin (IL)-6 blockade treatment. METHODS: We serially measured serum LRG levels in four s-JIA patients treated with the anti-IL-6 receptor antibody tocilizumab and determined the correlation between clinical symptoms and other inflammatory biomarkers and proinflammatory cytokines, including IL-18, IL-6, neopterin, and tumor necrosis factor-α receptor type I and II...
January 17, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28088248/the-spectrum-of-rheumatic-in-patient-diagnoses-at-a-pediatric-hospital-in-kenya
#17
Angela Migowa, Inés Colmegna, Carol Hitchon, Eugene Were, Evelyn Ng'ang'a, Thomas Ngwiri, John Wachira, Sasha Bernatsky, Rosie Scuccimarri
BACKGROUND: Pediatric rheumatic diseases are chronic illnesses that can cause considerable disease burden to children and their families. There is limited epidemiologic data on these diseases in East Africa. The aim of this study was to assess the spectrum of pediatric rheumatic diagnoses in an in-patient setting and determine the accuracy of ICD-10 codes in identifying these conditions. METHODS: Medical records from Gertrude's Children's Hospital in Kenya were reviewed for patients diagnosed with "diseases of the musculoskeletal system and connective tissue" as per ICD-10 diagnostic codes assigned at discharge between January and December 2011...
January 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28056736/investigation-of-associations-between-autoimmunity-associated-variants-in-pdcd1-and-juvenile-idiopathic-arthritis
#18
Christina I Tejeda, K Alaine Broadaway, Milton R Brown, Lori A Ponder, Michael J Ombrello, Mina Rohani Pichavant, Gabriel Wang, Sheila T Angeles-Han, Aimee O Hersh, John F Bohnsack, Karen N Conneely, Michael P Epstein, Sampath Prahalad
PURPOSE: Variants in the gene encoding Programmed Cell Death-1 (PDCD1) have been associated with susceptibility to Systemic Lupus Erythematosus and other autoimmune diseases. Given that clinically distinct autoimmune phenotypes share common genetic susceptibility factors, variants in PDCD-1 were tested for a possible association with Juvenile Idiopathic Arthritis (JIA). METHODS: Four single nucleotide polymorphisms (SNPS) in the PDCD1 gene were genotyped and analyzed: rs10204525, rs7568402, rs7421861 and rs11568821 in 834 cases and 855 self-identified Caucasian controls...
January 4, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28025136/cd3-cd56-natural-killer-t-cell-activity-in-children-with-different-forms-of-juvenile-idiopathic-arthritis-and-the-influence-of-etanercept-treatment-on-polyarticular-subgroup
#19
Juan Zhou, Yuan Ding, Yu Zhang, Ye Feng, Xuemei Tang, Xiaodong Zhao
Juvenile idiopathic arthritis (JIA) has three major onset types with widely varying clinical features. We assessed the natural killer T (NKT) cell function in patients with different JIA subtypes, and found systemic patients exhibited lower NKT cell counts, perforin and granzyme B expression, while the pauciarticular and polyarticular patients displayed higher perforin and granzyme B expression as compared with the controls. The synovial fluid had more NKT cells with higher levels of perforin, granzyme B, and tumour necrosis factor (TNF)-α than peripheral cells...
December 24, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27999545/il-1-inhibition-in-systemic-juvenile-idiopathic-arthritis
#20
REVIEW
Gabriella Giancane, Francesca Minoia, Sergio Davì, Giulia Bracciolini, Alessandro Consolaro, Angelo Ravelli
Systemic juvenile idiopathic arthritis (sJIA) is the form of childhood arthritis whose treatment is most challenging. The demonstration of the prominent involvement of interleukin (IL)-1 in disease pathogenesis has provided the rationale for the treatment with biologic medications that antagonize this cytokine. The three IL-1 blockers that have been tested so far (anakinra, canakinumab, and rilonacept) have all been proven effective and safe, although only canakinumab is currently approved for use in sJIA. The studies on IL-1 inhibition in sJIA published in the past few years suggest that children with fewer affected joints, higher neutrophil count, younger age at disease onset, shorter disease duration, or, possibly, higher ferritin level may respond better to anti-IL-1 treatment...
2016: Frontiers in Pharmacology
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