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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/29901756/introduction-of-a-novel-magnetic-resonance-imaging-based-scoring-system-for-assessing-disease-activity-in-children-with-juvenile-dermatomyositis
#1
Mandela Thyoka, Oba Adekunle, Clarissa Pilkington, Stephen Walters, Owen J Arthurs, Paul Humphries, Karl Johnson, Jeannette Kraft, Caren Landes, Thara Persaud, Raj Sinha, Amaka C Offiah
Objectives: We aimed to develop and assess the reliability of a novel MRI-based scoring system for reporting the severity of MRI findings in children with suspected JDM. Methods: Nine consultant paediatric radiologists independently assessed and scored 40 axial and 30 coronal thigh MR images of children with suspected JDM on two occasions using the juvenile dermatomyositis magnetic resonance Imaging Score (JIS). JIS was calculated for both reads for each plane and each limb, with possible scores ranging from 0 (normal) to 100 (severe)...
June 12, 2018: Rheumatology
https://www.readbyqxmd.com/read/29773272/juvenile-dermatomyositis-latest-advances
#2
REVIEW
Qiong Wu, Lucy R Wedderburn, Liza J McCann
Registries and biobanks for juvenile dermatomyositis (JDM) have generated statistical power to help understand pathogenesis and determine treatment and long-term outcomes in this rare and heterogeneous disease. Genotype, autoantibodies, muscle histology and early clinical features may predict prognosis and guide personalised treatment. While corticosteroids and disease-modifying anti-rheumatic drugs improve outcomes, there remain children who experience refractory disease. Ongoing research into the aberrant immune response and novel biological targets is necessary...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773266/genetic-interferonopathies-an-overview
#3
REVIEW
Despina Eleftheriou, Paul A Brogan
Interferonopathies comprise an expanding group of monogenic diseases characterised by disturbance of the homeostatic control of interferon (IFN)-mediated immune responses. Although differing in the degree of phenotypic expression and severity, the clinical presentation of these diseases shows a considerable degree of overlap, reflecting their common pathogenetic mechanisms. Increased understanding of the molecular basis of these Mendelian disorders has led to the identification of targeted therapies for these diseases, which could also be of potential relevance for non-genetic IFN-mediated diseases such as systemic lupus erythematosus and juvenile dermatomyositis...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773230/medications-received-by-patients-with-juvenile-dermatomyositis
#4
Takayuki Kishi, Nastaran Bayat, Michael M Ward, Adam M Huber, Lan Wu, Gulnara Mamyrova, Ira N Targoff, William J Warren-Hicks, Frederick W Miller, Lisa G Rider
OBJECTIVE: Few controlled studies are available to guide treatment decisions in juvenile dermatomyositis (JDM). This study evaluated therapies received, changes of treatment over time, and factors associated with medication choices in JDM. METHODS: We performed a retrospective analysis of the number and type of therapies and duration of treatment for 320 patients with JDM enrolled in a North American registry. Kaplan-Meier and logistic regression analysis were used to assess the association of demographic and clinical features and autoantibodies with medication usage...
March 28, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29770465/expression-of-myxovirus-resistance-protein-a-a-possible-marker-of-muscle-disease-activity-and-autoantibody-specificities-in-juvenile-dermatomyositis
#5
Sirisucha Soponkanaporn, Claire T Deakin, Peter W Schutz, Lucy R Marshall, Shireena A Yasin, Cerise M Johnson, Erdal Sag, Sarah L Tansley, Neil J McHugh, Lucy R Wedderburn, Thomas S Jacques
AIMS: To evaluate the relationship between expression of Myxovirus-resistance protein A (MxA) protein on muscle biopsies by immunohistochemistry and disease activity in JDM patients. Also, another aim was to investigate whether the expression of MxA is related with myositis-specific autoantibodies (MSA) status in JDM patients. METHODS: 103 patients (median aged 6.3, IQR 0.5-15.9) enrolled in the Juvenile Dermatomyositis Cohort and Biomarker Study (JDCBS). Muscle biopsies were stained with MxA and scored...
May 16, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29721695/inverse-gottron-papules-in-juvenile-dermatomyositis-an-under-recognized-clinical-entity
#6
Ankur Kumar Jindal, Sandesh Guleria, Rakesh Kumar Pilania, Anuradha Bishnoi, Keshavamurthy Vinay, Sunil Dogra, Deepti Suri, Amit Rawat, Surjit Singh
The objective of this study was to report four children having juvenile dermatomyositis (JDM) with inverse Gottron sign along with a brief review of literature of similarly published cases. This is a retrospective review of case records of all children diagnosed with JDM at a single tertiary care centre in North India. Children who were found to have Gottron papules over palmar surface were identified. A total of 127 children were diagnosed with JDM in the last 24 years (1994-2017). Out of these 127 patients, 4 were found to have Gottron papules over palmar surface...
May 2, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29720802/a-viral-polymyositis-masquerade-life-threatening-case-of-juvenile-dermatomyositis-complicated-by-systemic-capillary-leak-syndrome
#7
Bhaskara P Shelley, Shrijeet Chakraborti
This is a case report of an 8-year-old boy who developed an atypical, rare subphenotype of autoimmune inflammatory acute juvenile dermatomyositis (JDM), initially masquerading as viral polymyositis (PM)-like presentation, that was complicated by a hitherto unreported fulminant, life-threatening pediatric systemic capillary leak syndrome (SCLS). We highlight the close differential between viral PM and JDM, the baffling clinical syndromic constellation of hypotension with hemoconcentration - a "shock"-like syndrome, hypoalbuminemia without albuminuria, and generalized edema with the atypical JDM presentation, and stress crucial need to implement early aggressive, multipronged immunomodulatory treatment along with intensive fluid resuscitation which saved the life, this patient from a stormy, and turbulent 4-week clinical illness...
January 2018: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/29698985/-juvenile-dermatomyositis
#8
Sarah Keller, Amir Golsari, Jin Yamamura
No abstract text is available yet for this article.
April 26, 2018: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
https://www.readbyqxmd.com/read/29673367/comparing-the-importance-of-quality-measurement-themes-in-juvenile-idiopathic-inflammatory-myositis-between-patients-and-families-and-healthcare-professionals
#9
Heather O Tory, Ruy Carrasco, Thomas Griffin, Adam M Huber, Philip Kahn, Angela Byun Robinson, David Zurakowski, Susan Kim
BACKGROUND: A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. METHODS: Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA...
April 19, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29672804/imaging-features-of-the-juvenile-inflammatory-arthropathies
#10
Iwona Sudoł-Szopińska, Lennart Jans, Anne Grethe Jurik, Robert Hemke, Iris Eshed, Nathalie Boutry
We discuss the imaging of several juvenile inflammatory arthropathies including juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis, and chronic recurrent multifocal osteomyelitis. Juvenile idiopathic arthritis is the most common autoimmune chronic systemic disease of connective tissue in children. The remaining systemic juvenile connective tissue diseases are rare. However, they require early diagnosis and initiation of treatment to prevent injury, not only to the musculoskeletal system but also to the internal organs, and even death...
April 2018: Seminars in Musculoskeletal Radiology
https://www.readbyqxmd.com/read/29651119/update-on-outcome-assessment-in-myositis
#11
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...
May 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29610677/potential-association-of-lmna-associated-generalized-lipodystrophy-with-juvenile-dermatomyositis
#12
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
#13
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
#14
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
#15
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
#16
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 the 25 patients (72%) were positive for MSAs; seven with anti-melanoma differentiation associated gene (MDA) 5 (28%), five with anti-transcriptional intermediary factor (TIF)-1γ (20%), four with anti-MJ/nuclear matrix protein (NXP)-2 (16%), two with anti-Jo-1 (8%), one with anti- HMG-CoA reductase, one with anti-signal recognition peptide (SRP) antibodies (4% each), including co-existence and transition of MSAs in one patient each...
April 9, 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
#17
(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
#18
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
#19
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
#20
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)...
May 1, 2018: Rheumatology
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