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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/29457995/validation-of-a-brazilian-portuguese-version-of-the-transition-readiness-assessment-questionnaire-traq-in-a-population-of-transitional-youth-with-chronic-rheumatologic-disorders
#1
Catherine G Anelli, Claudio A Len, Maria T Terreri, Gleice C S Russo, Andreas O Reiff
OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool...
February 16, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29457372/clinical-characteristics-and-factors-associated-with-disability-and-impaired-quality-of-life-in-children-with-juvenile-systemic-sclerosis
#2
Brandi E Stevens, Kathryn S Torok, Suzanne C Li, Nicole Hershey, Megan Curran, Gloria C Higgins, Katharine F Moore, C Egla Rabinovich, Samuel Dodson, Anne M Stevens
OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry. METHODS: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics...
February 18, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29411095/-myositis-specific-antibodies-associated-with-juvenile-dermatomyositis
#3
K Eising, J Peitz, N Unterwalder, C Meisel, G Horneff
BACKGROUND: Juvenile dermatomyositis (JDM) is a rare autoimmune disease associated with typical skin changes and muscle weakness. Within the framework of the diagnostics, myositis-associated (MAA) and myositis-specific antibodies (MSA) can be detected. These are important for the assessment of the course of the disease and the prognosis. METHOD: In this study we searched for MAA and MSA by means of a line immunoassay in 12 currently supervised JDM patients in the Rheumatism Center Sankt Augustin...
February 6, 2018: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/29373707/cardiovascular-and-cerebrovascular-comorbidities-of-juvenile-dermatomyositis-in-us-children-an-analysis-of-the-national-inpatient-sample
#4
Jonathan I Silverberg, Lauren Kwa, Michael C Kwa, Anne E Laumann, Kaveh Ardalan
Objective: JDM is associated with multiple potential risk factors for cardiovascular disease, including reduced heart rate variability, systolic/diastolic cardiac dysfunction, abnormal brachial artery reactivity and metabolic syndrome. However, little is known about cardiovascular risk in JDM. We sought to examine the association between JDM and cardiovascular risk factors and disease in US children. Methods: Data from the 2002-12 National Inpatient Sample was analysed, including ∼20% of all US hospitalizations (n = 14 535 620 paediatric hospitalizations)...
January 24, 2018: Rheumatology
https://www.readbyqxmd.com/read/29367373/extensive-skin-ulcers-in-a-child-with-juvenile-dermatomyositis
#5
Aman Gupta, Rakesh Kumar Pilania, Venkata Durga Prasad, Sandesh Guleria
Juvenile dermatomyositis (JDM) is a multisystemic disorder. Vasculitic ulcers in JDM have been reported to involve axilla, elbow or extensor surfaces of other joints. We report a young boy with JDM who presented with extensive cutaneous ulcers involving scrotum, prepuce, gluteal region, neck, bilateral axilla, periumbilical area and bilateral elbows and popliteal fossa. His disease course was marked by several relapses and he required immunosuppression with prednisolone, subcutaneous methotrexate and intravenous cyclophosphamide...
January 23, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29363513/anti-nt5c1a-autoantibodies-are-associated-with-more-severe-disease-in-patients-with-juvenile-myositis
#6
Richard M Yeker, Iago Pinal-Fernandez, Takayuki Kishi, Katherine Pak, Ira N Targoff, Frederick W Miller, Lisa G Rider, Andrew L Mammen
OBJECTIVES: Autoantibodies recognising cytosolic 5'-nucleotidase 1A (NT5C1A) are found in adult patients with myositis and other autoimmune diseases. They are especially prevalent in adults with inclusion body myositis (IBM), in which they are associated with more severe weakness and higher mortality. This study was undertaken to define the prevalence and clinical features associated with anti-NT5C1A autoantibodies in juvenile myositis. METHODS: We screened sera from 380 patients with juvenile myositis, 30 patients with juvenile idiopathic arthritis (JIA) and 92 healthy control children for anti-NT5C1A autoantibodies...
January 23, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29361142/multiple-target-autoantigens-on-endothelial-cells-identified-in-juvenile-dermatomyositis-using-proteomics
#7
Rie Karasawa, Mayumi Tamaki, Toshiko Sato, Megumi Tanaka, Makiko Nawa, Kazuo Yudoh, James N Jarvis
Objective: Although generally classified within the group of inflammatory myopathies, JDM displays many pathological features of vasculitis. Previous work has shown that AECA are abundant in other forms of vasculitis. We therefore investigated whether such antibodies might also be detected in JDM. Methods: We screened plasma from children with JDM for the presence of AECA by western blotting and 2D gel electrophoresis (2DE) using proteins extracted from human aortic endothelial cells as the substrate...
January 18, 2018: Rheumatology
https://www.readbyqxmd.com/read/29342499/efficacy-and-safety-of-cyclophosphamide-treatment-in-severe-juvenile-dermatomyositis-shown-by-marginal-structural-modelling
#8
Claire T Deakin, Raquel Campanilho-Marques, Stefania Simou, Elena Moraitis, Lucy R Wedderburn, Eleanor Pullenayegum, Clarissa A Pilkington
OBJECTIVE: In patients with severe or refractory juvenile dermatomyositis (JDM), second-line treatments may be required. Cyclophosphamide (CYC) is used to treat some connective tissue diseases, but evidence of efficacy in JDM is limited. This study aimed to describe clinical improvement in JDM patients treated with CYC and model efficacy of CYC compared to patients not treated with CYC. METHODS: Clinical data on skin, global and muscle disease were analyzed from patients recruited to the Juvenile Dermatomyositis Cohort and Biomarker Study...
January 17, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29333701/the-childhood-arthritis-rheumatology-research-alliance-consensus-treatment-plans-towards-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#9
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/29314651/prevalence-and-predictors-of-asymptomatic-vertebral-fractures-in-inflammatory-myositis
#10
Latika Gupta, Able Lawrence, Sukesh Edavalath, Ramnath Misra
AIM: To assess the frequency and risk factors of asymptomatic vertebral fractures in inflammatory myositis. PATIENTS AND METHODS: Dorsal and lumbar spine lateral radiographs were taken for adults with inflammatory myositis and scored using Genant's semi-quantitative technique. Demographic data, weight, height, postmenopausal status, duration of corticosteroid use, drug intake, co-morbidities and past history of fractures were recorded. Bone mineral density (BMD) was assessed using dual-energy X-ray absorptiometry (DEXA)...
January 5, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29245175/development-and-testing-of-a-hybrid-measure-of-muscle-strength-in-juvenile-dermatomyositis-for-use-in-routine-care
#11
Giulia Camilla Varnier, Silvia Rosina, Cristina Ferrari, Angela Pistorio, Alessandro Consolaro, Francesca Bovis, Sara Dalprà, Clarissa Pilkington, Susan Maillard, Adele Civino, Elena Tsitsami, Jaime de Inocencio, Marija Jelusic, Jelena Vojinovic, Graciela Espada, Balahan Makay, Maria Martha Katsicas, Polixeni Pratsidou-Gertsi, Dragana Lazarevic, Anand Prahalad Rao, Denise Pires Marafon, Nicolino Ruperto, Alberto Martini, Angelo Ravelli
OBJECTIVE: To develop and test a hybrid measure of muscle strength for juvenile dermatomyositis (JDM), which is based on the combination of the Manual Muscle Testing 8 (MMT8) and the Childhood Myositis Assessment Scale (CMAS), but is more comprehensive than the former and more feasible than the latter. METHODS: The hybrid MMT/CMAS (hMC) is composed of all 8 items of the MMT8 and 3 items of the CMAS: 1) time of head lift; 2) assessment of abdominal muscles; 3) floor rise...
December 15, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29177080/eular-acr-classification-criteria-for-adult-and-juvenile-idiopathic-inflammatory-myopathies-and-their-major-subgroups-a-methodology-report
#12
Matteo Bottai, Anna Tjärnlund, Giola Santoni, Victoria P Werth, Clarissa Pilkington, Marianne de Visser, Lars Alfredsson, Anthony A Amato, Richard J Barohn, Matthew H Liang, Jasvinder A Singh, Rohit Aggarwal, Snjolaug Arnardottir, Hector Chinoy, Robert G Cooper, Katalin Danko, Mazen M Dimachkie, Brian M Feldman, Ignacio García-De La Torre, Patrick Gordon, Taichi Hayashi, James D Katz, Hitoshi Kohsaka, Peter A Lachenbruch, Bianca A Lang, Yuhui Li, Chester V Oddis, Marzena Olesinka, Ann M Reed, Lidia Rutkowska-Sak, Helga Sanner, Albert Selva-O'Callaghan, Yeong Wook Song, Jiri Vencovsky, Steven R Ytterberg, Frederick W Miller, Lisa G Rider, Ingrid E Lundberg
Objective: To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs) and their major subgroups. Methods: An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for inclusion in the criteria. Rheumatology, dermatology, neurology and paediatric clinics worldwide collected data on 976 IIM cases (74% adults, 26% children) and 624 non-IIM comparator cases with mimicking conditions (82% adults, 18% children)...
2017: RMD Open
https://www.readbyqxmd.com/read/29170979/predictors-of-changes-in-disease-activity-among-children-with-juvenile-dermatomyositis-enrolled-in-the-childhood-arthritis-and-rheumatology-research-alliance-carra-legacy-registry
#13
Divya Challa, Cynthia S Crowson, Timothy B Niewold, Ann M Reed
Determinants of changes in disease activity among patients with juvenile dermatomyositis (JDM) are unknown. Our objective was to develop predictive models to predict changes in disease activity using the CARRA Legacy Registry. The CARRA Legacy Registry included 658 subjects with definite or probably JDM with 297 subjects with a one follow-up visit after baseline, and we studied the 65 subjects with active disease at baseline. Linear regression models were used to build risk scores for changes in disease activity adjusted for baseline disease activity, age, sex, and disease duration...
November 23, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29106061/2017-european-league-against-rheumatism-american-college-of-rheumatology-classification-criteria-for-adult-and-juvenile-idiopathic-inflammatory-myopathies-and-their-major-subgroups
#14
Ingrid E Lundberg, Anna Tjärnlund, Matteo Bottai, Victoria P Werth, Clarissa Pilkington, Marianne de Visser, Lars Alfredsson, Anthony A Amato, Richard J Barohn, Matthew H Liang, Jasvinder A Singh, Rohit Aggarwal, Snjolaug Arnardottir, Hector Chinoy, Robert G Cooper, Katalin Dankó, Mazen M Dimachkie, Brian M Feldman, Ignacio Garcia-De La Torre, Patrick Gordon, Taichi Hayashi, James D Katz, Hitoshi Kohsaka, Peter A Lachenbruch, Bianca A Lang, Yuhui Li, Chester V Oddis, Marzena Olesinska, Ann M Reed, Lidia Rutkowska-Sak, Helga Sanner, Albert Selva-O'Callaghan, Yeong-Wook Song, Jiri Vencovsky, Steven R Ytterberg, Frederick W Miller, Lisa G Rider
OBJECTIVE: To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. METHODS: Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology, and pediatric clinics worldwide. Several statistical methods were utilized to derive the classification criteria. RESULTS: Based on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived...
October 27, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29089059/effective-induction-therapy-for-anti-srp-associated-myositis-in-childhood-a-small-case-series-and-review-of-the-literature
#15
E L Binns, E Moraitis, S Maillard, S Tansley, N McHugh, T S Jacques, L R Wedderburn, C Pilkington, S A Yasin, K Nistala
BACKGROUND: Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation. CASE PRESENTATIONS: Three new patients are reported. All 3 cases presented with profound, rapid-onset, proximal myopathy and markedly raised CK, but no rash...
October 31, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29079590/2017-european-league-against-rheumatism-american-college-of-rheumatology-classification-criteria-for-adult-and-juvenile-idiopathic-inflammatory-myopathies-and-their-major-subgroups
#16
Ingrid E Lundberg, Anna Tjärnlund, Matteo Bottai, Victoria P Werth, Clarissa Pilkington, Marianne de Visser, Lars Alfredsson, Anthony A Amato, Richard J Barohn, Matthew H Liang, Jasvinder A Singh, Rohit Aggarwal, Snjolaug Arnardottir, Hector Chinoy, Robert G Cooper, Katalin Dankó, Mazen M Dimachkie, Brian M Feldman, Ignacio Garcia-De La Torre, Patrick Gordon, Taichi Hayashi, James D Katz, Hitoshi Kohsaka, Peter A Lachenbruch, Bianca A Lang, Yuhui Li, Chester V Oddis, Marzena Olesinska, Ann M Reed, Lidia Rutkowska-Sak, Helga Sanner, Albert Selva-O'Callaghan, Yeong-Wook Song, Jiri Vencovsky, Steven R Ytterberg, Frederick W Miller, Lisa G Rider
OBJECTIVE: To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. METHODS: Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology and paediatric clinics worldwide. Several statistical methods were used to derive the classification criteria. RESULTS: Based on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived...
December 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29037322/improvement-of-nailfold-capillary-microangiopathy-after-immunosuppressant-therapy-in-a-child-with-clinically-amyopathic-juvenile-dermatomyositis
#17
Lúcia Maria Arruda Campos, Adriana M E Sallum, Cintia Z Camargo, Luís Eduardo C Andrade, Cristiane Kayser
No abstract text is available yet for this article.
September 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29037319/devic-s-disease-in-an-adolescent-girl-with-juvenile-dermatomyositis
#18
Melissa Mariti Fraga, Enedina Maria Lobato de Oliveira, Claudio Arnaldo Len, Maria Fernanda Campos, Maria Teresa Terreri
No abstract text is available yet for this article.
September 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29029283/trajectories-of-cardiorespiratory-fitness-in-patients-with-juvenile-dermatomyositis
#19
Kiki J Blom, Tim Takken, Barbara C H Huijgen, Judith Wienke, Annet van Royen-Kerkhof, Marco van Brussel
Objectives: Previous research demonstrated decreased cardiorespiratory fitness (CRF) in patients with JDM during active disease and remission. However, longitudinal data regarding trajectories of CRF are currently lacking. The objective of this study was to determine trajectories of CRF in patients with both monocyclic and chronic JDM, and to identify potential predictors of these trajectories. Methods: Thirty-six patients with JDM [median age (interquartile range) at diagnosis: 8...
September 27, 2017: Rheumatology
https://www.readbyqxmd.com/read/28977549/2016-acr-eular-adult-dermatomyositis-and-polymyositis-and-juvenile-dermatomyositis-response-criteria-methodological-aspects
#20
Lisa G Rider, Nicolino Ruperto, Angela Pistorio, Brian Erman, Nastaran Bayat, Peter A Lachenbruch, Howard Rockette, Brian M Feldman, Adam M Huber, Paul Hansen, Chester V Oddis, Ingrid E Lundberg, Anthony A Amato, Hector Chinoy, Robert G Cooper, Lorinda Chung, Katalin Danko, David Fiorentino, Ignacio García-De la Torre, Ann M Reed, Yeong Wook Song, Rolando Cimaz, Rubén J Cuttica, Clarissa A Pilkington, Alberto Martini, Janjaap van der Net, Susan Maillard, Frederick W Miller, Jiri Vencovsky, Rohit Aggarwal
Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM. Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions...
November 1, 2017: Rheumatology
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