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Systemic juvenile idiopathic arthritis

Fiona Moghaddas, Dominic De Nardo, Paul Baker, Lavinia Gordon, Simon Sadedin, Alicia Oshlack, Jonathan Akikusa, Roger Allen, Jane Munro, Justine Ellis, Seth Masters
No abstract text is available yet for this article.
February 2016: Pathology
Alessandro Consolaro, Esi M Morgan, Gabriella Giancane, Silvia Rosina, Stefano Lanni, Angelo Ravelli
Information technology in paediatric rheumatology has seen several exciting developments in recent years. The new multidimensional questionnaires for juvenile idiopathic arthritis, juvenile dermatomyositis, and juvenile autoinflammatory diseases integrate all major parent- and child-reported outcomes (PCROs) used in these diseases into a single tool, and provide an effective guide to manage, document change in health, assess effectiveness of therapeutic interventions, and verify the parent and child satisfaction with illness outcome...
September 2016: Clinical and Experimental Rheumatology
Filipa Oliveira-Ramos, Mónica Eusébio, Fernando M Martins, Ana Filipa Mourão, Carolina Furtado, Raquel Campanilho-Marques, Inês Cordeiro, Joana Ferreira, Marcos Cerqueira, Ricardo Figueira, Iva Brito, Helena Canhão, Maria José Santos, José A Melo-Gomes, João Eurico Fonseca
OBJECTIVES: To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. METHODS: Patients with JIA registered on the Rheumatic Diseases Portuguese Register ( older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index-articular (JADI-A) and Juvenile Arthritis Damage Index-extra-articular (JADI-E) damage index and disease activity were analysed...
2016: RMD Open
Anne-Mieke J W Haasnoot, Lenneke A Vernie, Aniki Rothova, Patricia V D Doe, Leonoor I Los, Nicoline E Schalij-Delfos, Joke H de Boer
BACKGROUND: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze uveitis activity, complications and visual prognosis in adulthood. METHODS: In this multicenter study, 67 adult patients (129 affected eyes) with JIA-uveitis were retrospectively studied for best corrected visual acuity, visual fields, uveitis activity, topical/systemic treatments, ocular complications, and ocular surgeries during their 18th, 22nd and 30th year of life...
2016: PloS One
Jennifer J Schoch, Stanislav N Tolkachjov, Jonathan A Cappel, Lawrence E Gibson, Dawn Marie R Davis
BACKGROUND/OBJECTIVES: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis rarely seen in children. Its features have not been well characterized in children. We sought to characterize the clinical features, etiologic associations, and treatment of PG in children younger than 18 years. METHODS: We performed a retrospective review of children younger than 18 years with PG at the Mayo Clinic from January 1976 to August 2013. RESULTS: Thirteen children with PG were identified (n = 8; 62% female)...
October 4, 2016: Pediatric Dermatology
Karen Put, Jessica Vandenhaute, Anneleen Avau, Annemarie Van Nieuwenhuijze, Ellen Brisse, Tim Dierckx, Omer Rutgeerts, Josselyn Garcia-Perez, Jaan Toelen, Mark Waer, Georges Leclercq, An Goris, Johan Van Weyenbergh, Adrian Liston, Lien de Somer, Carine Wouters, Patrick Matthys
OBJECTIVE: Systemic juvenile idiopathic arthritis (sJIA) is an immune-inflammatory disease characterized by arthritis and systemic features. The role of natural killer (NK) cells in sJIA pathogenesis remains unclear. Therefore, we performed a comprehensive analysis of NK cell phenotype and functionality in sJIA patients. METHODS: NK cell-specific transcriptional alterations were investigated by RNA-sequencing of highly purified NK cells from six active sJIA patients and six healthy controls...
October 1, 2016: Arthritis & Rheumatology
Wildéa Lice de Carvalho Jennings Pereira, Edna Maria Vissoci Reiche, Ana Paula Kallaur, Sayonara Rangel Oliveira, Andréa Name Colado Simão, Marcell Alysson Batisti Lozovoy, Lucas José Vaz Schiavão, Paula Raquel do Vale Pascoal Rodrigues, Daniela Frizon Alfieri, Tamires Flauzino, Damacio Ramón Kaimen-Maciel
OBJECTIVE: The aim of this study was to report the frequency of autoimmune disorders and autoantibodies in 22 patients with neuromyelitis optica (NMO), as well as whether the seropositivity for autoantibodies differs between anti-aquaporin 4 (AQP4) positive and AQP4 negative NMO patients. METHODS: Demographic, medical records, and a profile of autoantibodies were evaluated in 22 NMO patients, including AQP4, anti-thyroid-stimulating hormone receptor, antinuclear antibodies (ANA), anti-thyroperoxidase (anti-TPO), anti-thyroglobulin (anti-Tg), anti-double-stranded DNA, anti-neutrophil cytoplasmic, anti-cyclic citrullinate peptide, rheumatoid factor, anti-SSA/Ro, anti-SSB/La, anti-Smith antibodies (anti-Sm), anti-ribonucleoprotein, anti-nucleosome, and anti-Scl70...
October 3, 2016: Acta Neuropsychiatrica
Iwona Sudoł-Szopińska, Elżbieta Grochowska, Piotr Gietka, Mateusz Płaza, Grzegorz Pracoń, Fadhil Saied, Marta Walentowska-Janowicz
Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals in the developmental age. Radiography, which was described in the first part of this publication, is the standard modality in the assessment of this condition. Ultrasound and magnetic resonance imaging enable early detection of the disease which affects soft tissues, as well as bones. Ultrasound assessment involves: joint cavities, tendon sheaths and bursae for the presence of synovitis, intraand extraarticular fat tissue to visualize signs of inflammation, hyaline cartilage, cartilaginous epiphysis and subchondral bone to detect cysts and erosions, and ligaments, tendons and their entheses for signs of enthesopathies and tendinopathies...
September 2016: Journal of Ultrasonography
Iwona Sudoł-Szopińska, Genowefa Matuszewska, Piotr Gietka, Mateusz Płaza, Marta Walentowska-Janowicz
Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals at the developmental age. Radiography is the primary modality employed in the diagnostic imaging in order to identify changes typical of this disease entity and rule out other bone-related pathologies, such as neoplasms, posttraumatic changes, developmental defects and other forms of arthritis. The standard procedure involves the performance of comparative joint radiographs in two planes...
September 2016: Journal of Ultrasonography
Jiang Lijiao, L U Meiping, Guo Li, W U Jianqiang, Zou Lixia, X U Yiping
Objective: To investigate the serum levels of Th1/Th2 cytokines in children with non-systemic juvenile onset idiopathic arthritis (non-SOJIA). Methods: Clinical data of 41 children with non-SOJIA, including 11 cases of polyarthritis, 10 cases of oligoarthritis and 20 cases of enthesitis related JIA (ERA), admitted in Children's Hospital of Zhejiang University School of Medicine during November 2012 and May 2015 were retrospectively analyzed. Serum levels of Th1/Th2 cytokines including IL-2, IL-4, IL-6, IL-10, TNF-α and IFN-γ were measured by flow cytometry in patients with non-SOJIA, and compared with those in patients with SOJIA (SOJIA group, n=85) and healthy children (control group, n=202); their correlations with erythrocyte sedimentation rate and C reactive protein and CRP were analyzed...
May 25, 2016: Zhejiang da Xue Xue Bao. Yi Xue Ban, Journal of Zhejiang University. Medical Sciences
Anouk Verwoerd, Nienke M Ter Haar, Sytze de Roock, Sebastiaan J Vastert, Debby Bogaert
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The microbiome has received increasing attention as a potential contributing factor to the development of a wide array of immune-mediated diseases, including inflammatory bowel disease, type 1 diabetes and rheumatoid arthritis...
September 20, 2016: Pediatric Rheumatology Online Journal
Mario Abinun, Jonathan P Lane, Mark Wood, Mark Friswell, Terence J Flood, Helen E Foster
Severe infections are emerging as major risk factors for death among children with juvenile idiopathic arthritis (JIA). In particular, children with refractory JIA treated with long-term, multiple, and often combined immunosuppressive and antiinflammatory agents, including the new biological disease-modifying antirheumatic drugs (DMARDs), are at increased risk for severe infections and death. We investigated 4 persons with JIA who died during 1994-2013, three of overwhelming central venous catheter-related bacterial sepsis caused by coagulase-negative Staphylococus or α-hemolytic Streptococcus infection and 1 of disseminated adenovirus and Epstein-Barr virus infection)...
October 2016: Emerging Infectious Diseases
Christoph Tappeiner, Marina Mesquida, Alfredo Adán, Jordi Anton, Athimalaipet V Ramanan, Ester Carreno, Friederike Mackensen, Kaisu Kotaniemi, Joke H de Boer, Rosa Bou, Carmen García de Vicuña, Arnd Heiligenhaus
OBJECTIVE: To report on experience using the anti-interleukin 6 receptor antibody tocilizumab (TCZ) to treat severe and therapy-refractory uveitis associated with juvenile idiopathic arthritis (JIA). METHODS: Retrospective data were gathered from patients with JIA receiving TCZ treatment for uveitis. JIA and related uveitis data (disease onset, activity, structural complications, and topical and systemic antiinflammatory treatment) were evaluated at the start of TCZ (baseline) and every 3 months during TCZ therapy...
September 15, 2016: Journal of Rheumatology
Ana Paula Sakamoto, Clovis Artur Silva, Mariana Paes Leme Ferriani, Rosa Maria Rodrigues Pereira, Eloisa Bonfá, Claudia Saad-Magalhães, Eunice Okuda, Simone Appenzeller, Francisco Hugo Gomes, Ana Luiza Garcia Cunha, Mirna Henriques Tomich Salume, Daniela Petry Piotto, Maria Teresa Terreri
Chronic arthritis (CA) is an unusual condition in childhood-onset systemic lupus erythematosus (cSLE) and data in children is very limited. The aim of the study is to assess CA in a large population of cSLE patients, in a multicenter cross-sectional study including 852 cSLE patients followed in ten Pediatric Rheumatology referral services in state of São Paulo, Brazil. CA was observed in 32/852 (3.7 %) cSLE patients mostly in hands and ankles. Chronic monoarthritis was diagnosed in four cSLE patients, oligoarthritis in nine and polyarthritis in 19...
September 14, 2016: Rheumatology International
Lazaros I Sakkas
Systemic sclerosis (SSc) is a multisystem disease characterized by extensive collagen deposition in skin and internal organs, fibrointimal microvasculopathy, and activation of the immune system. T cells and B cells can promote fibrosis in SSc. Interleukin (IL)-6 is implicated in the pathogenesis of SSc. IL-6 is increased in the peripheral blood and lesional skin from patients with SSc, and induces fibroblast collagen production directly and indirectly by inducing profibrotic M2 macrophages. IL-6 also induces Th17 differentiation and promotes B cell differentiation toward Ig-producing plasma cells...
2016: Drug Design, Development and Therapy
Hessam Tabeian, Astrid D Bakker, Teun J de Vries, Behrouz Zandieh-Doulabi, Frank Lobbezoo, Vincent Everts
OBJECTIVE: The temporomandibular joint (TMJ) consists of three cartilaginous structures: the fossa, disc, and condyle. In juvenile idiopathic arthritis (JIA), inflammation of the TMJ leads to destruction of the condyle, but not of the fossa or the disc. Such a different effect of inflammation might be related to differences in matrix composition of the cartilaginous structures. METHODS: The matrix composition of the three TMJ structures was analyzed in juvenile porcine samples and in an in vitro system of cells isolated from each anatomical structure embedded in 3% agarose gels...
August 30, 2016: Archives of Oral Biology
Andrew S Zeft, Sampath Prahalad, Rayfel Schneider, Fatma Dedeoglu, Pamela F Weiss, Alexei A Grom, Carter Mix, C Arden Pope Rd
OBJECTIVES: Fine particulate matter (PM2.5) is a measurable component of ambient pollution, and positive associations of short-term PM2.5 exposure with the clinical presentation of systemic onset juvenile idiopathic arthritis (SJIA) in young children have been described in a regional cohort. Our objective was to further establish associations between short-term pollution exposures and the reported clinical event of SJIA onset in cases residing from multiple metropolitan regions. METHODS: A case-crossover study design was used to analyse associations of short-term PM2...
September 2016: Clinical and Experimental Rheumatology
Christelle Domngang Noche, Giles Kagmeni, Viola Dohvoma, Assumpta Lucienne Bella, Come Ebana Mvogo, Madeleine Singwe-Ngandeu
PURPOSE: To determine the profile of ophthalmic manifestations in chronic inflammatory rheumatic diseases (CIRD). METHODS: Observational study at the Yaounde Central Hospital and Innel Medical Centre (2004 to 2012). RESULTS: The study population (n = 36) consisted of 14 men and 22 women with average age of 47.9 ± 17.2 years. Cases of CIRD were rheumatoid arthritis (n = 16), systemic lupus erythematosus (n = 8), ankylosing spondylitis (n = 8), mixed connective tissue disease (n = 2), scleroderma (n = 1), and juvenile idiopathic arthritis (n = 1)...
September 6, 2016: Ocular Immunology and Inflammation
Mikel Alberdi-Saugstrup, Susan Nielsen, Pernille Mathiessen, Claus Henrik Nielsen, Klaus Müller
Two thirds of patients with juvenile idiopathic arthritis (JIA) treated with tumor necrosis factor (TNF)-alpha inhibitors respond initially, but only about one third of patients achieve clinical remission at follow-up. We evaluated the 1-year response and long-term treatment adherence to TNF inhibitor treatment in JIA patients naive to biologics and investigated if baseline myeloid-related protein (MRP)-8/14 and C-reactive protein (CRP) were predictive of treatment response. One hundred fifty-two patients were included in a unicenter observational, prospective study from 2002 to 2015, excluding patients with systemic-onset JIA...
August 25, 2016: Clinical Rheumatology
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