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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/28610606/biologic-therapies-for-refractory-juvenile-dermatomyositis-five-years-of-experience-of-the-childhood-arthritis-and-rheumatology-research-alliance-in-north-america
#1
C H Spencer, K Rouster-Stevens, H Gewanter, G Syverson, R Modica, K Schmidt, H Emery, C Wallace, S Grevich, K Nanda, Y D Zhao, S Shenoi, S Tarvin, S Hong, C Lindsley, J E Weiss, M Passo, K Ede, A Brown, K Ardalan, W Bernal, M L Stoll, B Lang, R Carrasco, C Agaiar, L Feller, H Bukulmez, R Vehe, H Kim, H Schmeling, D Gerstbacher, M Hoeltzel, B Eberhard, R Sundel, S Kim, A M Huber, A Patwardhan
BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA)...
June 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28589751/modelling-disease-activity-in-juvenile-dermatomyositis-a-bayesian-approach
#2
Eh Pieter van Dijkhuizen, Claire T Deakin, Lucy R Wedderburn, Maria De Iorio
Juvenile dermatomyositis is the most common form of the juvenile idiopathic inflammatory myopathies characterised by muscle and skin inflammation, leading to symmetric proximal muscle weakness and cutaneous symptoms. It has a fluctuating course and varying prognosis. In a Bayesian framework, we develop a joint model for four longitudinal outcomes, which accounts for within individual variability as well as inter-individual variability. Correlations among the outcome variables are introduced through a subject-specific random effect...
January 1, 2017: Statistical Methods in Medical Research
https://www.readbyqxmd.com/read/28550457/treatment-of-juvenile-dermatomyositis-an-update
#3
REVIEW
Charalampia Papadopoulou, Lucy R Wedderburn
The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response...
May 26, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28535894/association-between-demyelinating-disease-and-autoimmune-rheumatic-disease-in-a-pediatric-population
#4
Ana Luiza M Amorim, Nadia C Cabral, Fabiane M Osaku, Claudio A Len, Enedina M L Oliveira, Maria Teresa Terreri
INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients...
May 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28533552/physical-activity-for-paediatric-rheumatic-diseases-standing-up-against-old-paradigms
#5
REVIEW
Bruno Gualano, Eloisa Bonfa, Rosa M R Pereira, Clovis A Silva
Over the past 50 years it has become clear that physical inactivity is associated with chronic disease risk. For several rheumatic diseases, bed rest was traditionally advocated as the best treatment, but several levels of evidence support the imminent paradigm shift from the prescription of bed rest to physical activity in individuals with paediatric rheumatic diseases, in particular juvenile systemic lupus erythematosus, juvenile idiopathic arthritis, juvenile fibromyalgia, and juvenile dermatomyositis. Increasing levels of physical activity can alleviate several symptoms experienced by patients with paediatric rheumatic diseases, such as low aerobic fitness, pain, fatigue, muscle weakness and poor health-related quality of life...
May 23, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28514969/endothelial-progenitor-cell-number-is-not-decreased-in-34-children-with-juvenile-dermatomyositis-a-pilot-study
#6
Dong Xu, Akadia Kacha-Ochana, Gabrielle A Morgan, Chiang-Ching Huang, Lauren M Pachman
OBJECTIVE: A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM). METHODS: After obtaining informed consent, the EPC number from 34 fasting children with definite/probable JDM at various stages of therapy-initially untreated, active disease on medication and clinically inactive, off medication-was compared with 13 healthy fasting pediatric controls. The EPC number was determined by fluorescence activated cell sorting (FACS), CD34(+)/VEGFR2(+)/CD45dim(-), and assessed in conjunction with clinical variables: disease activity scores (DAS), duration of untreated disease (DUD), TNF-α allelic polymorphism (A/G) at the promoter region of -308, number of nailfold capillary end row loop (ERL) and von Willebrand factor antigen (vWF:Ag)...
May 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28448730/retinal-manifestations-of-juvenile-dermatomyositis-case-report-of-bilateral-diffuse-chorioretinopathy-with-paracentral-acute-middle-maculopathy-and-review-of-the-literature
#7
Rene Y Choi, Russell J Swan, Aimee Hersh, Albert T Vitale
PURPOSE: To review a case of bilateral diffuse chorioretinopathy as a presenting sign of juvenile dermatomyositis (JDM) and review the literature regarding retinal manifestations associated with this disease. METHODS: Review of case record and literature regarding retinal manifestations related to juvenile dermatomyositis. RESULTS: A 13-year-old girl presented with bilateral diffuse chorioretinopathy as the presenting sign of juvenile dermatomyositis...
April 27, 2017: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/28444299/environmental-factors-associated-with-disease-flare-in-juvenile-and-adult-dermatomyositis
#8
Gulnara Mamyrova, Lisa G Rider, Alison Ehrlich, Olcay Jones, Lauren M Pachman, Robert Nickeson, Lisa G Criscone-Schreiber, Lawrence K Jung, Frederick W Miller, James D Katz
Objective.: The aim was to assess environmental factors associated with disease flare in juvenile and adult dermatomyositis (DM). Methods.: An online survey of DM patients from the USA and Canada examined smoking, sun exposure, infections, medications, vaccines, stressful life events and physical activity during the 6 months before flares, or in the past 6 months in patients without flares. Differences were evaluated by χ 2 and Fisher's exact tests, and significant univariable results were examined in multivariable logistic regression...
April 21, 2017: Rheumatology
https://www.readbyqxmd.com/read/28434566/race-and-income-affects-outcomes-in-juvenile-dermatomyositis
#9
Philip J Hashkes
No abstract text is available yet for this article.
May 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28421601/evaluation-of-the-reliability-of-the-cutaneous-dermatomyositis-disease-area-and-severity-index-cdasi-and-the-cutaneous-assessment-tool-binary-method-cat-bm-in-juvenile-dermatomyositis-among-pediatric-dermatologists-rheumatologists-and-neurologists
#10
J Tiao, R Feng, E M Berger, J F Brandsema, C C Coughlin, N Khan, E A Kichula, M A Lerman, S Lvovich, P J McMahon, L G Rider, A I Rubin, L V Scalzi, D M Smith, A J Taxter, J R Treat, R P Williams, S W Yum, J Okawa, V P Werth
BACKGROUND: The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and Cutaneous Assessment Tool-Binary Method (CAT-BM) have been shown to be reliable and valid outcome measures to assess cutaneous disease in adult dermatomyositis (DM) and juvenile DM (JDM), respectively. OBJECTIVE: This study compared the CDASI and CAT-BM for use by pediatric dermatologists, pediatric rheumatologists, and pediatric neurologists in patients with JDM. METHODS: Five pediatric dermatologists, five pediatric rheumatologists, and five pediatric neurologists each evaluated 14 patients with JDM using the CDASI, CAT-BM, and skin Physician Global Assessment (PGA) scales...
April 19, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28412076/varicella-vaccination-elicits-a-humoral-and-cellular-response-in-children-with-rheumatic-diseases-using-immune-suppressive-treatment
#11
Noortje Groot, Gecilmara Pileggi, Cleonice B Sandoval, Ingrid Grein, Guy Berbers, Virginia Paes Leme Ferriani, Nico Wulffraat, Sytze de Roock
OBJECTIVE: To assess humoral and cellular responses to live-attenuated varicella zoster virus (VZV) vaccination of patients with juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) or juvenile scleroderma (JScle) compared to those of healthy controls (HC). METHODS: Before, 4-6weeks and one year after VZV vaccination, blood samples of patients and HC were collected. VZV-specific antibody concentrations were measured by ELISA and multiplex immune-assay...
May 15, 2017: Vaccine
https://www.readbyqxmd.com/read/28410093/race-income-and-disease-outcomes-in-juvenile-dermatomyositis
#12
Kathryn Phillippi, Mark Hoeltzel, Angela Byun Robinson, Susan Kim
OBJECTIVE: To determine the relationships among race, income, and disease outcomes in children with juvenile dermatomyositis (JDM). STUDY DESIGN: Data from 438 subjects with JDM enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were analyzed. Demographic data included age, sex, race, annual family income, and insurance status. Clinical outcomes included muscle strength, presence of rash, calcinosis, weakness, physical function, and quality of life measures...
May 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28403889/muscle-mri-at-the-time-of-questionable-disease-flares-in-juvenile-dermatomyositis-jdm
#13
Rabheh Abdul-Aziz, Chack-Yung Yu, Brent Adler, Sharon Bout-Tabaku, Katherine E Lintner, Melissa Moore-Clingenpeel, Charles H Spencer
BACKGROUND: The course of JDM has improved substantially over the last 70 years with early and aggressive treatments. Yet it remains difficult to detect disease flares as symptoms may be mild; signs of rash and muscle weakness vary widely and are often equivocal; laboratory tests of muscle enzyme levels are often normal; electromyography and muscle biopsy are invasive. Alternative tools are needed to help decide if more aggressive treatment is needed. Our objective is to determine the effectiveness of muscle Magnetic Resonance Imaging (MRI) in detecting JDM flares, and how an MRI affects physician's decision-making regarding treatment...
April 12, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28396024/coexisting-juvenile-dermatomyositis-and-sickle-cell-disease-maintaining-a-high-degree-of-suspicion
#14
Ellen Fraint, Maureen Leffler, Corinna L Schultz
Juvenile dermatomyositis is an idiopathic inflammatory myopathy of childhood not previously described in a patient with sickle cell disease. We present a case of an 11-year-old girl with sickle cell disease who was diagnosed subsequently with juvenile dermatomyositis, and highlight the diagnostic and therapeutic challenges of these concurrent chronic diseases.
April 7, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28385805/2016-american-college-of-rheumatology-european-league-against-rheumatism-criteria-for-minimal-moderate-and-major-clinical-response-in-adult-dermatomyositis-and-polymyositis-an-international-myositis-assessment-and-clinical-studies-group-paediatric-rheumatology
#15
Rohit Aggarwal, Lisa G Rider, Nicolino Ruperto, Nastaran Bayat, Brian Erman, Brian M Feldman, Chester V Oddis, Anthony A Amato, Hector Chinoy, Robert G Cooper, Maryam Dastmalchi, David Fiorentino, David Isenberg, James D Katz, Andrew Mammen, Marianne de Visser, Steven R Ytterberg, Ingrid E Lundberg, Lorinda Chung, Katalin Danko, Ignacio García-De la Torre, Yeong Wook Song, Luca Villa, Mariangela Rinaldi, Howard Rockette, Peter A Lachenbruch, Frederick W Miller, Jiri Vencovsky
To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM). Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. The PAPRIKA (Potentially All Pairwise Rankings of All Possible Alternatives) method determined the relative weights of core set measures and conjoint analysis definitions...
May 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28385804/2016-american-college-of-rheumatology-european-league-against-rheumatism-criteria-for-minimal-moderate-and-major-clinical-response-in-juvenile-dermatomyositis-an-international-myositis-assessment-and-clinical-studies-group-paediatric-rheumatology-international
#16
Lisa G Rider, Rohit Aggarwal, Angela Pistorio, Nastaran Bayat, Brian Erman, Brian M Feldman, Adam M Huber, Rolando Cimaz, Rubén J Cuttica, Sheila Knupp de Oliveira, Carol B Lindsley, Clarissa A Pilkington, Marilynn Punaro, Angelo Ravelli, Ann M Reed, Kelly Rouster-Stevens, Annet van Royen-Kerkhof, Frank Dressler, Claudia Saad Magalhaes, Tamás Constantin, Joyce E Davidson, Bo Magnusson, Ricardo Russo, Luca Villa, Mariangela Rinaldi, Howard Rockette, Peter A Lachenbruch, Frederick W Miller, Jiri Vencovsky, Nicolino Ruperto
To develop response criteria for juvenile dermatomyositis (DM). We analysed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial...
May 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28384112/inflammatory-myopathies-with-cutaneous-involvement-from-diagnosis-to-therapy
#17
Lyubomir A Dourmishev
The group of idiopathic inflammatory myopathies (IIM) include various disorders of skeletal muscles with or without skin involvement. The most common types are dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, scleromyositis, overlap or anti-synthetase syndromes, etc. Recently, numerous new antibodies defining the characteristic clinical phenotype have been described as anti-MDA5 antibodies associated with interstitial lung disease and amyopathic dermatomyositis or anti-TIF1γ antibodies as markers for paraneoplastic dermatomyositis...
March 1, 2017: Folia Medica
https://www.readbyqxmd.com/read/28382787/2016-american-college-of-rheumatology-european-league-against-rheumatism-criteria-for-minimal-moderate-and-major-clinical-response-in-adult-dermatomyositis-and-polymyositis-an-international-myositis-assessment-and-clinical-studies-group-paediatric-rheumatology
#18
Rohit Aggarwal, Lisa G Rider, Nicolino Ruperto, Nastaran Bayat, Brian Erman, Brian M Feldman, Chester V Oddis, Anthony A Amato, Hector Chinoy, Robert G Cooper, Maryam Dastmalchi, David Fiorentino, David Isenberg, James D Katz, Andrew Mammen, Marianne de Visser, Steven R Ytterberg, Ingrid E Lundberg, Lorinda Chung, Katalin Danko, Ignacio García-De la Torre, Yeong Wook Song, Luca Villa, Mariangela Rinaldi, Howard Rockette, Peter A Lachenbruch, Frederick W Miller, Jiri Vencovsky
OBJECTIVE: To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM). METHODS: Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. The PAPRIKA (Potentially All Pairwise Rankings of All Possible Alternatives) method determined the relative weights of core set measures and conjoint analysis definitions...
May 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28382778/2016-american-college-of-rheumatology-european-league-against-rheumatism-criteria-for-minimal-moderate-and-major-clinical-response-in-juvenile-dermatomyositis-an-international-myositis-assessment-and-clinical-studies-group-paediatric-rheumatology-international
#19
Lisa G Rider, Rohit Aggarwal, Angela Pistorio, Nastaran Bayat, Brian Erman, Brian M Feldman, Adam M Huber, Rolando Cimaz, Rubén J Cuttica, Sheila Knupp de Oliveira, Carol B Lindsley, Clarissa A Pilkington, Marilynn Punaro, Angelo Ravelli, Ann M Reed, Kelly Rouster-Stevens, Annet van Royen-Kerkhof, Frank Dressler, Claudia Saad Magalhaes, Tamás Constantin, Joyce E Davidson, Bo Magnusson, Ricardo Russo, Luca Villa, Mariangela Rinaldi, Howard Rockette, Peter A Lachenbruch, Frederick W Miller, Jiri Vencovsky, Nicolino Ruperto
OBJECTIVE: To develop response criteria for juvenile dermatomyositis (DM). METHODS: We analyzed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial...
April 6, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28365577/submaximal-exercise-capacity-in-juvenile-dermatomyositis-after-longterm-disease-the-contribution-of-muscle-lung-and-heart-involvement
#20
Kristin Schjander Berntsen, Anita Tollisen, Thomas Schwartz, Eva Kirkhus, Trond Mogens Aaløkken, May Brit Lund, Berit Flatø, Ivar Sjaastad, Helga Sanner
OBJECTIVE: To compare submaximal exercise capacity in patients with juvenile dermatomyositis (JDM) with controls, and analyze contributions of muscle, heart, and lung impairment in patients. METHODS: Fifty-nine patients with JDM, with a mean 16.9 years after symptom onset, and 59 sex- and age-matched controls completed a 6-min walk test (6MWT) and a timed up and go (TUG) test. Muscle function, disease activity/damage, and health-related quality of life (HRQOL) were assessed by validated tools; heart function by echocardiography and electrocardiography; and lung function by spirometry, DLCO, and body plethysmography...
April 1, 2017: Journal of Rheumatology
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