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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/28805016/microvascular-abnormalities-assessed-by-nailfold-capillaroscopy-in-juvenile-dermatomyositis-after-medium-to-long-term-follow-up
#1
Zoltan Barth, Birgit N Witczak, Berit Flatø, Akos Koller, Ivar Sjaastad, Helga Sanner
OBJECTIVE: In juvenile dermatomyositis (JDM), microvascular abnormalities measured by nailfold capillaroscopy (NFC) are common early in disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to longstanding JDM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. METHODS: Fifty-eight JDM patients clinically examined median 16.8 (2-38) years after disease onset were compared with matched controls...
August 13, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28794565/superimposed-segmental-manifestation-of-juvenile-amyopathic-dermatomyositis-in-a-9-year-old-boy
#2
Isil Bulur, Hilal Kaya Erdogan, Zeynep Nurhan Saracoglu, Rudolf Happle, Funda Canaz
Dermatomyositis is a well-known autoimmune disorder. On the other hand, juvenile amyopathic dermatomyositis (JADM) occurs rather rarely. Here, we report an unusual case in a 9-year-old Turkish boy showing a unilateral linear inflammatory skin lesion that was followed, after 16 months, by the appearance of bilateral disseminated features JADM.
July 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28776361/first-report-of-anti-tif1%C3%AE-dermatomyositis-in-a-patient-with-myelodysplastic-syndrome
#3
B Palterer, G Vitiello, D Cammelli
Inflammatory myopathies as para-neoplastic phenomena were first described by Sterz in 1916. Recently, myositis specific autoantibodies were described in cancer-associated myositis. Anti-transcription intermediary factor 1 gamma (anti-TIF1γ) antibodies have been found in both young adults affected by juvenile dermatomyositis and in elderly patients with cancer-associated myositis. In this regard, we report herein the first case of anti-TIF1γ dermatomyositis secondary to a myelodysplastic syndrome.
August 3, 2017: Reumatismo
https://www.readbyqxmd.com/read/28774458/ultrasound-in-arthritis
#4
REVIEW
Iwona Sudoł-Szopińska, Claudia Schueller-Weidekamm, Athena Plagou, James Teh
Ultrasound is currently performed in everyday rheumatologic practice. It is used for early diagnosis, to monitor treatment results, and to diagnose remission. The spectrum of pathologies seen in arthritis with ultrasound includes early inflammatory features and associated complications. This article discusses the spectrum of ultrasound features of arthritides seen in rheumatoid arthritis and other connective tissue diseases in adults, such as Sjögren syndrome, lupus erythematosus, dermatomyositis, polymyositis, and juvenile idiopathic arthritis...
September 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28763333/recent-clinical-trials-in-idiopathic-inflammatory-myopathies
#5
Valérie Leclair, Ingrid E Lundberg
PURPOSE OF REVIEW: Idiopathic inflammatory myopathies (IIMs) are complex multisystemic autoimmune diseases. Glucocorticoids remain the cornerstone of treatment in IIM, and the benefit of additional immunosuppressors is still debated. A limited number of controlled clinical trials have been available to support treatment guidelines, but in the last year, several clinical trials have been published. In this review, the highlights of recently published and on-going clinical trials in IIM will be summarized and discussed...
July 29, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28709855/subcutaneous-edema-in-juvenile-dermatomyositis
#6
Alfonso Ragnar Torres Jiménez, Eunice Solís-Vallejo, Adriana Ivonne Céspedes-Cruz, Magdalena Sánchez-Uribe
No abstract text is available yet for this article.
July 11, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28704993/a-case-of-juvenile-amyopathic-dermatomyositis
#7
Maddalena Napolitano, Matteo Megna, Lorenzo Squillace, Claudia Costa, Massimiliano Scalvenzi
No abstract text is available yet for this article.
July 11, 2017: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/28693511/cardiac-findings-in-children-with-juvenile-dermatomyositis-at-disease-presentation
#8
Serdar Cantez, Gil J Gross, Ian MacLusky, Brian M Feldman
BACKGROUND: Juvenile Dermatomyositis (JDM) is a pediatric vasculopathy characterized primarily by skin and muscle involvement. Cardiac findings have been reported in children with JDM but have rarely been investigated in detail. METHODS: We aimed to describe the relevant clinical and laboratory cardiac findings of a cohort of patients with JDM, followed at one centre, at disease diagnosis. RESULTS: We performed a retrospective review of 105 patients with JDM, followed from 1991 to 2007...
July 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28685324/phenotypic-characteristics-and-outcome-of-juvenile-dermatomyositis-in-arab-children
#9
Sulaiman M Al-Mayouf, Nora AlMutiari, Mohammed Muzaffer, Rawiah Shehata, Adel Al-Wahadneh, Reem Abdwani, Safia Al-Abrawi, Mohammed Abu-Shukair, Zeyad El-Habahbeh, Abdullah Alsonbul
This study describes the disease characteristics and outcome of Arab children with juvenile dermatomyositis (JDM) and compares the findings with other ethnicities. We retrospectively reviewed the hospital registries of the participating hospitals for children with JDM seen between 1990 and 2016 in three Arab countries. All patients fulfilled Bohan and Peter criteria for JDM, diagnosed before 14 years of age and were of Arab ethnicity. Clinical and laboratory features as well as the long-term outcomes including accrual disease damage were collected at the last follow-up visit...
July 6, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28610606/biologic-therapies-for-refractory-juvenile-dermatomyositis-five-years-of-experience-of-the-childhood-arthritis-and-rheumatology-research-alliance-in-north-america
#10
C H Spencer, K Rouster-Stevens, H Gewanter, G Syverson, R Modica, K Schmidt, H Emery, C Wallace, S Grevich, K Nanda, Y D Zhao, S Shenoi, S Tarvin, S Hong, C Lindsley, J E Weiss, M Passo, K Ede, A Brown, K Ardalan, W Bernal, M L Stoll, B Lang, R Carrasco, C Agaiar, L Feller, H Bukulmez, R Vehe, H Kim, H Schmeling, D Gerstbacher, M Hoeltzel, B Eberhard, R Sundel, S Kim, A M Huber, A Patwardhan
BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA)...
June 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28589751/modelling-disease-activity-in-juvenile-dermatomyositis-a-bayesian-approach
#11
Eh Pieter van Dijkhuizen, Claire T Deakin, Lucy R Wedderburn, Maria De Iorio
Juvenile dermatomyositis is the most common form of the juvenile idiopathic inflammatory myopathies characterised by muscle and skin inflammation, leading to symmetric proximal muscle weakness and cutaneous symptoms. It has a fluctuating course and varying prognosis. In a Bayesian framework, we develop a joint model for four longitudinal outcomes, which accounts for within individual variability as well as inter-individual variability. Correlations among the outcome variables are introduced through a subject-specific random effect...
January 1, 2017: Statistical Methods in Medical Research
https://www.readbyqxmd.com/read/28550457/treatment-of-juvenile-dermatomyositis-an-update
#12
REVIEW
Charalampia Papadopoulou, Lucy R Wedderburn
The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response...
May 26, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28535894/association-between-demyelinating-disease-and-autoimmune-rheumatic-disease-in-a-pediatric-population
#13
Ana Luiza M Amorim, Nadia C Cabral, Fabiane M Osaku, Claudio A Len, Enedina M L Oliveira, Maria Teresa Terreri
INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients...
May 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28533552/physical-activity-for-paediatric-rheumatic-diseases-standing-up-against-old-paradigms
#14
REVIEW
Bruno Gualano, Eloisa Bonfa, Rosa M R Pereira, Clovis A Silva
Over the past 50 years it has become clear that physical inactivity is associated with chronic disease risk. For several rheumatic diseases, bed rest was traditionally advocated as the best treatment, but several levels of evidence support the imminent paradigm shift from the prescription of bed rest to physical activity in individuals with paediatric rheumatic diseases, in particular juvenile systemic lupus erythematosus, juvenile idiopathic arthritis, juvenile fibromyalgia, and juvenile dermatomyositis. Increasing levels of physical activity can alleviate several symptoms experienced by patients with paediatric rheumatic diseases, such as low aerobic fitness, pain, fatigue, muscle weakness and poor health-related quality of life...
May 23, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28514969/endothelial-progenitor-cell-number-is-not-decreased-in-34-children-with-juvenile-dermatomyositis-a-pilot-study
#15
Dong Xu, Akadia Kacha-Ochana, Gabrielle A Morgan, Chiang-Ching Huang, Lauren M Pachman
OBJECTIVE: A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM). METHODS: After obtaining informed consent, the EPC number from 34 fasting children with definite/probable JDM at various stages of therapy-initially untreated, active disease on medication and clinically inactive, off medication-was compared with 13 healthy fasting pediatric controls. The EPC number was determined by fluorescence activated cell sorting (FACS), CD34(+)/VEGFR2(+)/CD45dim(-), and assessed in conjunction with clinical variables: disease activity scores (DAS), duration of untreated disease (DUD), TNF-α allelic polymorphism (A/G) at the promoter region of -308, number of nailfold capillary end row loop (ERL) and von Willebrand factor antigen (vWF:Ag)...
May 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28448730/retinal-manifestations-of-juvenile-dermatomyositis-case-report-of-bilateral-diffuse-chorioretinopathy-with-paracentral-acute-middle-maculopathy-and-review-of-the-literature
#16
Rene Y Choi, Russell J Swan, Aimee Hersh, Albert T Vitale
PURPOSE: To review a case of bilateral diffuse chorioretinopathy as a presenting sign of juvenile dermatomyositis (JDM) and review the literature regarding retinal manifestations associated with this disease. METHODS: Review of case record and literature regarding retinal manifestations related to juvenile dermatomyositis. RESULTS: A 13-year-old girl presented with bilateral diffuse chorioretinopathy as the presenting sign of juvenile dermatomyositis...
April 27, 2017: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/28444299/environmental-factors-associated-with-disease-flare-in-juvenile-and-adult-dermatomyositis
#17
Gulnara Mamyrova, Lisa G Rider, Alison Ehrlich, Olcay Jones, Lauren M Pachman, Robert Nickeson, Lisa G Criscone-Schreiber, Lawrence K Jung, Frederick W Miller, James D Katz
Objective.: The aim was to assess environmental factors associated with disease flare in juvenile and adult dermatomyositis (DM). Methods.: An online survey of DM patients from the USA and Canada examined smoking, sun exposure, infections, medications, vaccines, stressful life events and physical activity during the 6 months before flares, or in the past 6 months in patients without flares. Differences were evaluated by χ 2 and Fisher's exact tests, and significant univariable results were examined in multivariable logistic regression...
April 21, 2017: Rheumatology
https://www.readbyqxmd.com/read/28434566/race-and-income-affects-outcomes-in-juvenile-dermatomyositis
#18
Philip J Hashkes
No abstract text is available yet for this article.
May 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28421601/evaluation-of-the-reliability-of-the-cutaneous-dermatomyositis-disease-area-and-severity-index-cdasi-and-the-cutaneous-assessment-tool-binary-method-cat-bm-in-juvenile-dermatomyositis-among-pediatric-dermatologists-rheumatologists-and-neurologists
#19
J Tiao, R Feng, E M Berger, J F Brandsema, C C Coughlin, N Khan, E A Kichula, M A Lerman, S Lvovich, P J McMahon, L G Rider, A I Rubin, L V Scalzi, D M Smith, A J Taxter, J R Treat, R P Williams, S W Yum, J Okawa, V P Werth
BACKGROUND: The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and Cutaneous Assessment Tool-Binary Method (CAT-BM) have been shown to be reliable and valid outcome measures to assess cutaneous disease in adult dermatomyositis (DM) and juvenile DM (JDM), respectively. OBJECTIVE: This study compared the CDASI and CAT-BM for use by pediatric dermatologists, pediatric rheumatologists, and pediatric neurologists in patients with JDM. METHODS: Five pediatric dermatologists, five pediatric rheumatologists, and five pediatric neurologists each evaluated 14 patients with JDM using the CDASI, CAT-BM, and skin Physician Global Assessment (PGA) scales...
April 19, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28412076/varicella-vaccination-elicits-a-humoral-and-cellular-response-in-children-with-rheumatic-diseases-using-immune-suppressive-treatment
#20
Noortje Groot, Gecilmara Pileggi, Cleonice B Sandoval, Ingrid Grein, Guy Berbers, Virginia Paes Leme Ferriani, Nico Wulffraat, Sytze de Roock
OBJECTIVE: To assess humoral and cellular responses to live-attenuated varicella zoster virus (VZV) vaccination of patients with juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) or juvenile scleroderma (JScle) compared to those of healthy controls (HC). METHODS: Before, 4-6weeks and one year after VZV vaccination, blood samples of patients and HC were collected. VZV-specific antibody concentrations were measured by ELISA and multiplex immune-assay...
May 15, 2017: Vaccine
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