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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/28099216/a-rare-case-of-amyopathic-juvenile-dermatomyositis-associated-to-psoriasis-successfully-treated-with-ustekinumab
#1
Claudia L Montoya, Martha L Gonzalez, Fabio E Ospina, Gabriel J Tobón
Coexistence between two or more immunological-mediated diseases implies a clinical and therapeutic challenge that is uncommon in the clinical field. We report a rare case of a young patient with amyopathic juvenile dermatomyositis and erythrodermic psoriasis, with an excellent response to ustekinumab.
January 18, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28079914/intravenous-immunoglobulin-in-pediatric-rheumatology-when-to-use-it-and-what-is-the-evidence
#2
Martha M Rodriguez, Linda Wagner-Weiner
Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. " The literature supports the use of IVIG for juvenile dermatomyositis, although it is unclear whether its use should be limited to those children with more severe or refractory disease...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28077146/childhood-arthritis-and-rheumatology-research-alliance-consensus-clinical-treatment-plans-for-juvenile-dermatomyositis-with-skin-predominant-disease
#3
Susan Kim, Philip Kahn, Angela B Robinson, Bianca Lang, Andrew Shulman, Edward J Oberle, Kenneth Schikler, Megan Lea Curran, Lilliana Barillas-Arias, Charles H Spencer, Lisa G Rider, Adam M Huber
BACKGROUND: Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript...
January 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28073708/juvenile-dermatomyositis-positive-for-anti-dna-mismatch-repair-enzyme-antibodies
#4
Naoko Kubo, Koichi Yanaba, Sota Kikuchi, Osamu Fukuchi, Hidemi Nakagawa, Hiroyuki Namba, Yoshinao Muro
No abstract text is available yet for this article.
January 11, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28058540/juvenile-dermatomyositis-a-tertiary-center-experience
#5
Kenan Barut, Pinar Ozge Avar Aydin, Amra Adrovic, Sezgin Sahin, Ozgur Kasapcopur
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty...
January 5, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28050594/recurrence-of-juvenile-dermatomyositis-8%C3%A2-years-after-remission
#6
Ken Muramatsu, Hideyuki Ujiie, Mayumi Yokozeki, Ichiro Tsukinaga, Mai Ito, Takaaki Shikano, Akira Suzuki, Yusuke Tozawa, Ichiro Kobayashi
No abstract text is available yet for this article.
January 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28045754/anti-nxp2-positive-paraneoplastic-dermatomyositis-with-histopathologic-changes-confined-to-the-acrosyringia
#7
Jose Luis Ramírez-Bellver, Elena Macías, Claudia Bernárdez, Joaquín López-Robles, Maria Del Carmen Vegas-Sánchez, Jose Luis Díaz-Recuero, Hernán Quiceno, Luis Requena
BACKGROUND: Paraneoplastic syndromes consist of a group of disorders that are not related to the extension of the primary tumor or its metastases and that might be the first manifestation of a hidden neoplasm. It is a well-known association between dermatomyositis (DM) and cancer, especially gynecological tumors in women and lung cancer in men. METHODS: We describe the case of a 67-year-old male who developed muscular weakness and pruritic skin lesions. Skin biopsies were performed and histologic findings were consistent with DM...
January 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28004828/analysis-of-cxcr5-th17-cells-in-relation-to-disease-activity-and-tnf-inhibitor-therapy-in-rheumatoid-arthritis
#8
Deepika Singh, Matthew Henkel, Bernadette Sendon, June Feng, Anthony Fabio, Diana Metes, Larry W Moreland, Mandy J McGeachy
Th17 and TfH cells are thought to promote tissue inflammation and autoantibody production, respectively, in autoimmune diseases including rheumatoid arthritis (RA). TfH cells that co-express Th17 markers (CXCR5(+)Th17) encompass both of these pathogenic functions, and are increased in some human autoimmune settings including juvenile dermatomyositis. We investigated CXCR5(+)Th17 cells in RA subjects with stable or active disease and before and after TNF inhibitor therapy. CXCR5(+)Th17 cell frequency was increased in RA compared to healthy controls, but other helper T cell subsets were not different...
December 22, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27987494/-significance-of-glucose-6-phosphate-isomerase-assay-in-early-diagnosis-of-rheumatoid-arthritis
#9
J Xu, J Liu, L Zhu, X W Zhang, Z G Li
OBJECTIVE: To explore the titer of glucose-6-phosphate isomerase (GPI) for early diagnosis of the outpatient with rheumatoid arthritis (RA) in real life, and to analyze its relationship with disease activity. METHODS: In the study, 1 051 patients with arthritis were collected in the group who had joints tender and swelling, and 90 cases of healthy people as a control group. ELISA method was used to detect the serum level of GPI, and according to clinical features and laboratory test, all the patients including 525 RA patients, the other patients including osteoarthritis (OA), 134 cases of seronegative spine joint disease (SpA), 104 cases of systemic lupus erythematosus (SLE), 31 cases of primary Sjogren syndrome (pSS), 24 cases of gout arthritis (GA), 22 cases of other connective tissue diseases (including polymyalgia rheumatica, dermatomyositis, systemic sclerosis, adult Still disease) and 46 cases of other diseases (including 165 cases of osteoporosis, avascular necrosis of the femoral head, traumatic osteomyelitis, bone and joint disease, juvenile rheumatoid arthritis, tumor)...
December 18, 2016: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/27968927/autoimmune-collagen-vascular-diseases-kids-are-not-just-little-people
#10
Sean Timpane, Heather Brandling-Bennett, Arni K Kristjansson
Morphea, dermatomyositis (DM), and discoid lupus erythematosus (DLE) are autoimmune collagen vascular diseases that can present at any age. In all three of these diseases, the tenants of diagnosis and treatment are largely the same in both children and adults, with a few notable differences. Children with morphea are more likely to present with the linear subtype and have a higher incidence of extracutaneous manifestations. Children often need early aggressive systemic treatment to try to prevent long-term sequelae of morphea...
November 2016: Clinics in Dermatology
https://www.readbyqxmd.com/read/27943079/assessment-of-type-i-interferon-signaling-in-pediatric-inflammatory-disease
#11
Gillian I Rice, Isabelle Melki, Marie-Louise Frémond, Tracy A Briggs, Mathieu P Rodero, Naoki Kitabayashi, Anthony Oojageer, Brigitte Bader-Meunier, Alexandre Belot, Christine Bodemer, Pierre Quartier, Yanick J Crow
PURPOSE: Increased type I interferon is considered relevant to the pathology of a number of monogenic and complex disorders spanning pediatric rheumatology, neurology, and dermatology. However, no test exists in routine clinical practice to identify enhanced interferon signaling, thus limiting the ability to diagnose and monitor treatment of these diseases. Here, we set out to investigate the use of an assay measuring the expression of a panel of interferon-stimulated genes (ISGs) in children affected by a range of inflammatory diseases...
December 9, 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27837048/cutaneous-improvement-in-refractory-adult-and-juvenile-dermatomyositis-after-treatment-with-rituximab
#12
Rohit Aggarwal, Priyadarshini Loganathan, Diane Koontz, Zengbiao Qi, Ann M Reed, Chester V Oddis
OBJECTIVE: The aim was to assess the efficacy of rituximab for the cutaneous manifestations of adult DM and JDM. METHODS: Patients with refractory adult DM (n = 72) and JDM (n = 48) were treated with rituximab in a randomized placebo-phase-controlled trial [either rituximab early drug (week 0/1) or rituximab late arms (week 8/9), such that all subjects received study drug]. Stable concomitant therapy was allowed. Cutaneous disease activity was assessed using the Myositis Disease Activity Assessment Tool, which grades cutaneous disease activity on a visual analog scale...
November 11, 2016: Rheumatology
https://www.readbyqxmd.com/read/27835954/characteristics-and-outcome-of-children-with-juvenile-dermatomyositis-in-cape-town-a-cross-sectional-study
#13
Lawrence Owino Okong'o, Monika Esser, Jo Wilmshurst, Christiaan Scott
BACKGROUND: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. METHODS: We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa...
November 11, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27813167/how-often-are-pediatric-patients-with-clinically-amyopathic-dermatomyositis-truly-amyopathic
#14
Edward J Oberle, Michelle L Bayer, Yvonne E Chiu, Dominic O Co
BACKGROUND: Pediatric patients can present with skin manifestations of dermatomyositis without overt weakness (clinically amyopathic juvenile dermatomyositis [JDM]), but it is unclear how often this happens and how often they have subclinical muscle inflammation. OBJECTIVE: Our goal was to determine the frequency of clinically amyopathic JDM and the frequency with which a thorough evaluation uncovers subclinical myositis at a single institution. METHODS: A retrospective review was performed of 46 patients diagnosed with JDM at Children's Hospital of Wisconsin...
November 4, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27803137/validity-of-the-stage-of-exercise-scale-in-children-with-rheumatologic-conditions
#15
Samantha L Stephens, Mark S Tremblay, Guy Faulkner, Joseph Beyene, Tri H Nguyen, Suneye Koohsari, Elizaveta Limenis, Brian M Feldman
OBJECTIVE: To determine the face, content, and construct validity of the Stages of Exercise Scale (SOES) in children with rheumatologic conditions [juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM)], and if the validity of the SOES differs by disease type by comparing it with a disease control with a chronic respiratory illness [cystic fibrosis (CF)]. METHODS: Sixty-seven children and adolescents (43 female) ages 11 to 18 years with a diagnosis of either JDM (n = 15), JIA (n = 39), or CF (n = 13) completed the SOES; scales of sensibility, process of change, decisional balance, and self-efficacy; the Child Health Assessment Questionnaire; and patient/physician ratings of disease severity...
December 2016: Journal of Rheumatology
https://www.readbyqxmd.com/read/27803135/childhood-arthritis-and-rheumatology-research-alliance-consensus-clinical-treatment-plans-for-juvenile-dermatomyositis-with-persistent-skin-rash
#16
Adam M Huber, Susan Kim, Ann M Reed, Ruy Carrasco, Brian M Feldman, Sandy D Hong, Philip Kahn, Homaira Rahimi, Angela Byun Robinson, Richard K Vehe, Jennifer E Weiss, Charles Spencer
OBJECTIVE: Juvenile dermatomyositis (JDM) is the most common form of idiopathic inflammatory myopathy in children. While outcomes are generally thought to be good, persistence of skin rash is a common problem. The goal of this study was to describe the development of clinical treatment plans (CTP) for children with JDM characterized by persistent skin rash despite complete resolution of muscle involvement. METHODS: The Childhood Arthritis and Rheumatology Research Alliance, a North American consortium of pediatric rheumatologists and other healthcare providers, used a combination of Delphi surveys and nominal group consensus meetings to develop CTP that reflected consensus on typical treatments for patients with JDM with persistent skin rash...
January 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/27803134/gene-expression-profiling-in-blood-and-affected-muscle-tissues-reveals-differential-activation-pathways-in-patients-with-new-onset-juvenile-and-adult-dermatomyositis
#17
Consuelo M López De Padilla, Cynthia S Crowson, Molly S Hein, Richard S Pendegraft, Michael A Strausbauch, Timothy B Niewold, Floranne C Ernste, Erik Peterson, Emily C Baechler, Ann M Reed
OBJECTIVE: To identify shared and differential molecular pathways in blood and affected muscle between adult dermatomyositis (DM) and juvenile DM, and their association with clinical disease activity measures. METHODS: Gene expression of transcription factors and cytokines involved in differentiation and effector function of T cell subsets, regulatory T cells and follicular Th cells, were analyzed in the blood from 21 newly diagnosed adult and 26 juvenile DM subjects and in 15 muscle specimens (7 adult and 8 juvenile DM) using a custom RT2 Profiler PCR Array...
January 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/27784480/-role-of-magnetic-resonance-imaging-in-the-diagnosis-of-juvenile-dermato-myositis-and-polymyositis-in-chinese-children
#18
J M Lai, F Q Wu, Z X Zhou, X Y Yuan, G X Su, S N Li, Y C Yan, J Zhu, M Kang
Objective: To evaluate the utility of magnetic resonance imaging (MRI) in diagnosis of juvenile dermatomyositis and polymyositis (JDM-PM) in children. Method: Fifty-four patients with JDM-PM in the active stage were enrolled in the study group. Twelve patients with benign acute childhood myositis and forty patients with juvenile idiopathic arthritis (JIA) complicated with myositis were enrolled as controls. MRI imaging of thighs was performed in all patients, fast spin echo T1WI, T2WI, and STIR were obtained in all patients...
October 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27762188/information-technology-in-paediatric-rheumatology
#19
REVIEW
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
https://www.readbyqxmd.com/read/27752355/cardiac-involvement-in-adult-and-juvenile-idiopathic-inflammatory-myopathies
#20
REVIEW
Thomas Schwartz, Louise Pyndt Diederichsen, Ingrid E Lundberg, Ivar Sjaastad, Helga Sanner
Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention...
2016: RMD Open
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