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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/28290112/protecting-bone-health-in-pediatric-rheumatic-diseases-pharmacological-considerations
#1
REVIEW
Yujuan Zhang, Diana Milojevic
Bone health in children with rheumatic conditions may be compromised due to several factors related to the inflammatory disease state, delayed puberty, altered life style, including decreased physical activities, sun avoidance, suboptimal calcium and vitamin D intake, and medical treatments, mainly glucocorticoids and possibly some disease-modifying anti-rheumatic drugs. Low bone density or even fragility fractures could be asymptomatic; therefore, children with diseases of high inflammatory load, such as systemic onset juvenile idiopathic arthritis, juvenile dermatomyositis, systemic lupus erythematosus, and those requiring chronic glucocorticoids may benefit from routine screening of bone health...
March 13, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28251541/profile-of-pediatric-idiopathic-inflammatory-myopathies-from-a-tertiary-care-center-of-eastern-india
#2
Sumantra Sarkar, Tanushree Mondal, Arpan Saha, Rakesh Mondal, Supratim Datta
OBJECTIVES: To report data on Idiopathic inflammatory myopathies (IIM) from eastern India. METHODS: All IIM patients diagnosed over the last 5 y (2011-2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata. RESULTS: Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]...
March 2, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28208969/acute-presentation-of-juvenile-dermatomyositis-with-subclinical-cardiac-involvement-a-rare-case
#3
Rhythm Khera, Shrayash Khare, Shailendra Kumar Singh
Cardiac involvements are common in patients with Dermatomyositis, most of which are not severe enough to present definite or readily observable symptoms. However, Cardiovascular (CVS) manifestations constitute a major cause of death in these patients. The most frequently reported clinically evident of CVS manifestations in-patient of dermatomyositis are Congestive Heart Failure (CHF), conduction aberrations, that may predispose to complete heart block and coronary artery disease. The principal pathophysiological mechanisms that may produce these cardiac manifestations involve coronary artery disease as well as small vessels vasculitis of the myocardium...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28203172/recurrent-multiple-squamous-cell-carcinomas-on-the-scalp-in-a-patient-with-juvenile-dermatomyositis
#4
Yota Sato, Taku Fujimura, Yumi Kambayashi, Akira Tsukada, Takanori Hidaka, Kayo Tanita, Takahiro Haga, Akira Hashimoto, Setsuya Aiba
Both long-term administration of immunosuppressive agents and chronic inflammatory conditions, such as autoimmune disease, could be risk factors for the development of cutaneous squamous cell carcinoma (cSCC). In this report, we present a case of recurrent multiple cSCC on the scalp in a patient with juvenile dermatomyositis who had been administered cyclosporine and Predonine since she was a 1-year-old infant. Interestingly, immunohistochemical staining revealed IL-17-producing cells adjacent to IL-17R-expressing atypical keratinocytes...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28202739/anti-hmgcr-autoantibodies-in-juvenile-idiopathic-inflammatory-myopathies-identify-a-rare-but-clinically-important-subset-of-patients
#5
Sarah L Tansley, Zoe E Betteridge, Stefania Simou, Thomas S Jacques, Clarissa Pilkington, Mark Wood, Kishore Warrier, Lucy R Wedderburn, Neil J McHugh
OBJECTIVE: We aimed to establish the prevalence and clinical associations of anti-HMG-CoA-reductase (anti-HMGCR) in a large UK cohort with juvenile myositis. METHODS: There were 381 patients investigated for anti-HMGCR using ELISA. RESULTS: Anti-HMGCR autoantibodies were detected in 4 patients (1%). These children had no or minimal rash and significant muscle disease. Muscle biopsies were considered distinctive, with widespread variation in fiber size, necrotic fibers, and chronic inflammatory cell infiltrates; all had prolonged elevation of creatine kinase and all ultimately received biologic therapies...
February 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28174638/juvenile-dermatomyositis-in-a-4-year-old-kenyan-girl
#6
Marlous L Grijsen, Deborah Mchaile, Inge Geut, Raimos Olomi, Maitseo Nwako, Luis Requena, William P Howlett, Daudi R Mavura, Marieke C J Dekker
To our knowledge, this is the first case report of juvenile dermatomyositis (JDM) in Tanzania. It demonstrates that the characteristic cutaneous findings of JDM may easily be overlooked, especially on dark skin, and the difficulty of clinical management in resource-constrained settings.
February 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28158183/beyond-the-mhc-a-canine-model-of-dermatomyositis-shows-a-complex-pattern-of-genetic-risk-involving-novel-loci
#7
Jacquelyn M Evans, Rooksana E Noorai, Kate L Tsai, Alison N Starr-Moss, Cody M Hill, Kendall J Anderson, Thomas R Famula, Leigh Anne Clark
Juvenile dermatomyositis (JDM) is a chronic inflammatory myopathy and vasculopathy driven by genetic and environmental influences. Here, we investigated the genetic underpinnings of an analogous, spontaneous disease of dogs also termed dermatomyositis (DMS). As in JDM, we observed a significant association with a haplotype of the major histocompatibility complex (MHC) (DLA-DRB1*002:01/-DQA1*009:01/-DQB1*001:01), particularly in homozygosity (P-val = 0.0001). However, the high incidence of the haplotype among healthy dogs indicated that additional genetic risk factors are likely involved in disease progression...
February 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28143550/spectrum-of-paediatric-rheumatic-diseases-in-nigeria
#8
Babatunde Hakeem Olaosebikan, Olufemi Oladipo Adelowo, Barakat Adeola Animashaun, Richard Oluyinka Akintayo
BACKGROUND: Paediatric rheumatology service in Sub-Sahara African is virtually not available as there is a shortage of paediatric rheumatologists and other rheumatology health professionals. We aim to describe the clinical spectrum and the frequencies of paediatric rheumatic diseases (PRDs) in Lagos State University Teaching Hospital (LASUTH), Lagos, Nigeria. METHODS: This is a retrospective review of patients with PRDs seen over a five year period (March 2010 to February 2016) at the rheumatology clinic and children ward of LASUTH...
January 31, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28137405/contraception-for-adolescents-with-chronic-rheumatic-diseases
#9
Benito Lourenço, Katia T Kozu, Gabriela N Leal, Marco F Silva, Elisabeth G C Fernandes, Camila M P França, Fernando H C Souza, Clovis A Silva
Contraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE), antiphospholipid syndrome (APS), juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM). Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Combined oral contraceptives (COC) are strictly prohibited for JSLE and APS patients with positive antiphospholipid antibodies...
January 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28133992/the-family-impact-of-caring-for-a-child-with-juvenile-dermatomyositis
#10
Samantha Kountz-Edwards, Christa Aoki, Caitlin Gannon, Rowena Gomez, Matthew Cordova, Wendy Packman
Background Juvenile dermatomyositis (JDM), a rare autoimmune disease, accounts for more than 80% of idiopathic inflammatory myopathy childhood cases, making it the most common idiopathic inflammatory myopathy among children. The average age of onset is approximately 7 years and commonly leads a chronic course. Symptoms of JDM include cutaneous features (Gottron's rash, heliotrope rash, or nail fold capillary changes), musculoskeletal features, calcinosis and lipodystrophy (a symmetrical deficit of subcutaneous fatty tissue), and acanthosis (thickening of the skin)...
January 1, 2017: Chronic Illness
https://www.readbyqxmd.com/read/28129483/anti-3-hydroxy-3-methylglutaryl-coenzyme-a-reductase-autoantibodies-are-associated-with-drb1-07-01-and-severe-myositis-in-pediatric-myositis-patients
#11
Takayuki Kishi, Lisa G Rider, Katherine Pak, Lilliana Barillas-Arias, Michael Henrickson, Paul L McCarthy, Bracha Shaham, Pamela F Weiss, Iren Horkayne-Szakaly, Ira N Targoff, Frederick Miller, Andrew L Mammen
OBJECTIVE: Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of pediatric anti-HMGCR-positive myositis patients. METHODS: The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed...
January 27, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28099216/a-rare-case-of-amyopathic-juvenile-dermatomyositis-associated-to-psoriasis-successfully-treated-with-ustekinumab
#12
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
#13
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
#14
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
#15
Naoko Kubo, Koichi Yanaba, Sota Kikuchi, Osamu Fukuchi, Hidemi Nakagawa, Hiroyuki Namba, Yoshinao Muro
No abstract text is available yet for this article.
February 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28058540/juvenile-dermatomyositis-a-tertiary-center-experience
#16
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...
February 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28050594/recurrence-of-juvenile-dermatomyositis-8%C3%A2-years-after-remission
#17
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
#18
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
#19
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
#20
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
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