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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/29651119/update-on-outcome-assessment-in-myositis
#1
REVIEW
Lisa G Rider, Rohit Aggarwal, Pedro M Machado, Jean-Yves Hogrel, Ann M Reed, Lisa Christopher-Stine, Nicolino Ruperto
The adult and juvenile myositis syndromes, commonly referred to collectively as idiopathic inflammatory myopathies (IIMs), are systemic autoimmune diseases with the hallmarks of muscle weakness and inflammation. Validated, well-standardized measures to assess disease activity, known as core set measures, were developed by international networks of myositis researchers for use in clinical trials. Composite response criteria using weighted changes in the core set measures of disease activity were developed and validated for adult and juvenile patients with dermatomyositis and adult patients with polymyositis, with different thresholds for minimal, moderate and major improvement in adults and juveniles...
April 12, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29610677/potential-association-of-lmna-associated-generalized-lipodystrophy-with-juvenile-dermatomyositis
#2
Melis Sahinoz, Shafaq Khairi, Ashley Cuttitta, Graham F Brady, Amit Rupani, Rasimcan Meral, Marwan K Tayeh, Peedikayil Thomas, Meredith Riebschleger, Sandra Camelo-Piragua, Jeffrey W Innis, M Bishr Omary, Daniel E Michele, Elif A Oral
Background: Juvenile dermatomyositis (JDM) is an auto-immune muscle disease which presents with skin manifestations and muscle weakness. At least 10% of the patients with JDM present with acquired lipodystrophy. Laminopathies are caused by mutations in the lamin genes and cover a wide spectrum of diseases including muscular dystrophies and lipodystrophy. The p.T10I LMNA variant is associated with a phenotype of generalized lipodystrophy that has also been called atypical progeroid syndrome...
2018: Clinical Diabetes and Endocrinology
https://www.readbyqxmd.com/read/29595280/-idiopathic-inflammatory-myopathies
#3
Jiří Vencovský
Idiopathic inflammatory myopathies form a heterogeneous group of acquired inflammatory diseases afflicting striated muscles. The disease is frequently accompanied by systemic and organ involvement. Dermatomyositis, polymyositis, cancer associated myositis, immune mediated necrotizing myopathy, myositis in overlap syndromes, juvenile myositis and inclusion body myositis are the most frequently encountered subtypes. The basic manifestation is usually painless muscle weakness brought about by inflammation and by other immune changes at the impacted muscles...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29576174/advances-in-juvenile-dermatomyositis-myositis-specific-antibodies-aid-in-understanding-disease-heterogeneity
#4
Lauren M Pachman, Amer M Khojah
No abstract text is available yet for this article.
April 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29561468/scrotal-and-penile-ulcer-in-juvenile-dermatomyositis-an-unusual-occurrence
#5
Ankur Kumar Jindal, Avinash Sharma, Amit Rawat
No abstract text is available yet for this article.
March 21, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29532710/myositis-specific-autoantibodies-in-japanese-patients-with-juvenile-idiopathic-inflammatory-myopathies
#6
Masahiro Ueki, Ichiro Kobayashi, Shunichiro Takezaki, Yusuke Tozawa, Yuka Okura, Masafumi Yamada, Masataka Kuwana, Tadashi Ariga
OBJECTIVES: The aim of our study is to clarify the association of myositis-specific autoantibodies (MSAs) with clinical and laboratory features in Japanese patients with juvenile idiopathic inflammatory myopathies (JIIMs). METHODS: We retrospectively analyzed the frequency of MSAs and their association with clinical or laboratory findings in 25 Japanese patients with JIIMs in Hokkaido district. RESULTS: Eighteen of 25 patients (72%) were positive for MSAs; seven with anti-melanoma differentiation associated gene (MDA) 5 (28%), 5 with anti-transcriptional intermediary factor (TIF)-1γ (20%), 4 with anti-MJ/nuclear matrix protein (NXP)-2 (16%), 2 with anti-Jo-1 (8%), 1 with anti- HMG-CoA reductase, 1 with anti-signal recognition peptide (SRP) antibodies (4% each), including co-existence and transition of MSAs in one patient each...
March 13, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29530915/correction-2016-american-college-of-rheumatology-european-league-against-rheumatism-criteria-for-minimal-moderate-and-major-clinical-response-in-juvenile-dermatomyositis
#7
(no author information available yet)
No abstract text is available yet for this article.
April 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29520367/architectural-b-cell-organization-in-skeletal-muscle-identifies-subtypes-of-dermatomyositis
#8
Josefine Radke, Randi Koll, Corinna Preuße, Debora Pehl, Kremena Todorova, Constanze Schönemann, Yves Allenbach, Eleonora Aronica, Marianne de Visser, Frank L Heppner, Joachim Weis, Soroush Doostkam, Thierry Maisonobe, Olivier Benveniste, Hans-Hilmar Goebel, Werner Stenzel
Objective: To study the B-cell content, organization, and existence of distinct B-cell subpopulations in relation to the expression of type 1 interferon signature related genes in dermatomyositis (DM). Methods: Evaluation of skeletal muscle biopsies from patients with adult DM (aDM) and juvenile DM (jDM) by histology, immunohistochemistry, electron microscopy, and quantitative reverse-transcription PCR. Results: We defined 3 aDM subgroups-classic (containing occasional B cells without clusters), B-cell-rich, and follicle-like aDM-further elucidating IM B-lymphocyte maturation and immunity...
May 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29496916/koebner-phenomenon-in-juvenile-dermatomyositis
#9
W James Tidwell, Jeffrey P Callen
No abstract text is available yet for this article.
March 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29474663/muscle-ischaemia-associated-with-nxp2-autoantibodies-a-severe-subtype-of-juvenile-dermatomyositis
#10
Jessie Aouizerate, Marie De Antonio, Brigitte Bader-Meunier, Christine Barnerias, Christine Bodemer, Arnaud Isapof, Pierre Quartier, Isabelle Melki, Jean-Luc Charuel, Guillaume Bassez, Isabelle Desguerre, Romain K Gherardi, François-Jérôme Authier, Cyril Gitiaux
Objectives: Myositis-specific autoantibodies (MSAs) are increasingly used to delineate distinct subgroups of JDM. The aim of our study was to explore without a priori hypotheses whether MSAs are associated with distinct clinical-pathological changes and severity in a monocentric JDM cohort. Methods: Clinical, biological and histological findings from 23 JDM patients were assessed. Twenty-six histopathological parameters were subjected to multivariate analysis. Results: Autoantibodies included anti-NXP2 (9/23), anti-TIF1γ (4/23), anti-MDA5 (2/23), no MSAs (8/23)...
February 20, 2018: Rheumatology
https://www.readbyqxmd.com/read/29462840/a-bayesian-semiparametric-markov-regression-model-for-juvenile-dermatomyositis
#11
Maria De Iorio, Natacha Gallot, Beatriz Valcarcel, Lucy Wedderburn
Juvenile dermatomyositis (JDM) is a rare autoimmune disease that may lead to serious complications, even to death. We develop a 2-state Markov regression model in a Bayesian framework to characterise disease progression in JDM over time and gain a better understanding of the factors influencing disease risk. The transition probabilities between disease and remission state (and vice versa) are a function of time-homogeneous and time-varying covariates. These latter types of covariates are introduced in the model through a latent health state function, which describes patient-specific health over time and accounts for variability among patients...
February 20, 2018: Statistics in Medicine
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
#12
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
#13
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
#14
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
#15
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)...
April 1, 2018: Rheumatology
https://www.readbyqxmd.com/read/29367373/extensive-skin-ulcers-in-a-child-with-juvenile-dermatomyositis
#16
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
#17
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...
May 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29361142/multiple-target-autoantigens-on-endothelial-cells-identified-in-juvenile-dermatomyositis-using-proteomics
#18
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...
April 1, 2018: Rheumatology
https://www.readbyqxmd.com/read/29342499/efficacy-and-safety-of-cyclophosphamide-treatment-in-severe-juvenile-dermatomyositis-shown-by-marginal-structural-modelling
#19
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-and-rheumatology-research-alliance-consensus-treatment-plans-toward-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#20
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 methods. Innovative comparative effectiveness approaches are needed to efficiently study treatment strategies 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
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