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Dermatomyositis childhood

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...
September 28, 2016: Revista Brasileira de Reumatologia
Fabian Speth, Johannes-Peter Haas, Claas H Hinze
BACKGROUND: High-dose intravenous immune globulins (IVIg) are frequently used in refractory juvenile dermatomyositis (JDM) but are often poorly tolerated. High-dose recombinant human hyaluronidase-facilitated subcutaneous immune globulins (fSCIg) allow the administration of much higher doses of immune globulins than conventional subcutaneous immune globulin therapy and may be an alternative to IVIg. The safety and efficacy of fSCIg therapy in JDM is unknown. CASE PRESENTATION: In this retrospective case series, five patients with steroid-refractory severe JDM were treated with high-dose fSCIg due to IVIg adverse effects (severe headaches, nausea, vomiting, difficult venous access)...
2016: Pediatric Rheumatology Online Journal
Eun Jin Doh, Jungyoon Moon, Sue Shin, Soo Hyun Seo, Hyun Sun Park, Hyun-Sun Yoon, Soyun Cho
Calcinosis is rarely observed in juvenile-onset amyopathic dermatomyositis in contrast to juvenile-onset dermatomyositis. A 6-year-old female presented with several 0.5 to 2 cm-sized painless grouped masses on both knees for 3 years. The patient also presented with multiple erythematous scaly patches and plaques on both elbows, knuckles, buttock, ankles and cheeks. Her mother had similar skin lesions which were erythematous scaly patches on the knuckles and elbows, since her childhood. When skin biopsy was performed from a left knee nodule, liquid chalky discharge was observed...
June 2016: Annals of Dermatology
Lúcia Maria Arruda Campos, Adriana M E Sallum, Cintia Z Camargo, Luís Eduardo C Andrade, Cristiane Kayser
Clinically amyopathic dermatomyositis (CADM) is an extremely rare entity in childhood and only 75 cases have been described in the literature. These patients present mild or no muscle involvement, while skin involvement is similar to that seen in classic dermatomyositis (DM). Systemic inflammatory vasculopathy is a hallmark of juvenile dermatomyositis (JDM), especially affecting the microcirculation. Nailfold capillaroscopy (NFC) is a non-invasive method that allows direct visualization of nailfold capillary loops around the proximal edge of the finger nails...
April 18, 2016: Revista Brasileira de Reumatologia
N Hendel, M K Akmatov, J Hamel, C Vogelberg, F Pessler
We aimed to evaluate the fraction of exhaled nitric oxide (FENO50) and deaerated exhaled breath condensate pH (dEBCpH) as non-invasive markers of subclinical airway inflammation in pediatric patients with rheumatologic disorders. We determined FENO50 and dEBCpH in a prospective study spanning at least 12 months, comprising 85 pediatric patients with rheumatologic disorders, including juvenile idiopathic arthritis (JIA, n  =  63), chronic recurrent multifocal osteomyelitis (CRMO, n  =  6), systemic lupus erythematosus (SLE, n  =  3), juvenile dermatomyositis (JDM, n  =  1) and other rheumatic disorders (n  =  12)...
June 2016: Journal of Breath Research
Adam M Huber, Leanne M Ward
Childhood rheumatic diseases are associated with negative impacts on the skeleton, related to both the underlying illness and complications of therapy. The effects of medications like corticosteroids are well recognized, leading to reductions in bone mineral density and bone strength and concomitant increases in bone fragility and fracture risk. The impact of factors directly attributable to the underlying disease is not as well recognized. In this article, we review relevant literature to identify data which can contribute to an understanding of the impact of childhood rheumatic disease on the skeleton...
August 2016: Seminars in Arthritis and Rheumatism
G Esther A Habers, G J F Joyce Bos, Annet van Royen-Kerkhof, Otto T H M Lelieveld, Wineke Armbrust, Tim Takken, Marco van Brussel
OBJECTIVE: To study for the first time in a randomized controlled trial the feasibility, safety and efficacy of an exercise training programme in children and adolescents with JDM. METHODS: Patients were randomly assigned to the Intervention Group (IG; n = 14) or Waiting Control Group (WCG; n = 12). The intervention comprised an individually tailored 12-week home-based exercise programme of treadmill interval training and strength exercises. The efficacy of the IG over usual care (WCG) was examined with mixed linear regression (intention-to-treat)...
July 2016: Rheumatology
Sampath Prahalad, Courtney E McCracken, Lori A Ponder, Sheila T Angeles-Han, Kelly A Rouster Stevens, Larry B Vogler, Carl D Langefeld, Susan D Thompson
BACKGROUND: Clinically distinct autoimmune phenotypes share genetic susceptibility factors. We investigated the prevalence of familial autoimmunity among subjects with juvenile idiopathic arthritis (JIA), childhood systemic lupus erythematosus (cSLE) and juvenile dermatomyositis (JDM) in the CARRA Registry, the largest multicenter observational Registry for pediatric rheumatic disease. METHODS: Children with JIA, cSLE and JDM enrolled in the CARRA Registry between May 2010 and May 2012 were investigated for differences in proportion of subjects who had first-degree relatives (FDR) with autoimmunity...
2016: Pediatric Rheumatology Online Journal
Raquel Campanilho-Marques, Beverley Almeida, Claire Deakin, Katie Arnold, Natacha Gallot, Maria de Iorio, Kiran Nistala, Clarissa A Pilkington, Lucy R Wedderburn
OBJECTIVE: To compare the abbreviated Cutaneous Assessment Tool (CAT), Disease Activity Score (DAS), and Myositis Intention to Treat Activity Index (MITAX) and correlate them with the physician's 10-cm skin visual analog scale (VAS) in order to define which tool best assesses skin disease in patients with juvenile dermatomyositis. METHODS: A total of 71 patients recruited to the UK Juvenile Dermatomyositis Cohort and Biomarker Study were included and assessed for skin disease using the CAT, DAS, MITAX, and skin VAS...
October 2016: Arthritis Care & Research
Dong Xu, Chiang-Ching Huang, Akadia Kachaochana, Gabrielle A Morgan, Maria F Bonaldo, Marcelo B Soares, Fabricio Costa, John Sarwark, Simone T Sredni, Lauren M Pachman
OBJECTIVE: To identify differentially expressed microRNA (miRNA) in muscle biopsies (MBx) from 15 untreated children with juvenile dermatomyositis (JDM) compared with 5 controls. METHODS: Following MBx miRNA profiling, differentially expressed miRNA and their protein targets were validated by quantitative real-time PCR (qRT-PCR) and immunological assay. The association of miRNA-10a and miRNA-10b with clinical data was evaluated, including Disease Activity Score (DAS), von Willebrand factor antigen (vWF:Ag), nailfold capillary end row loops, duration of untreated disease, and tumor necrosis factor (TNF)-α-308A allele...
January 2016: Journal of Rheumatology
Liza J McCann, Clare E Pain
Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications...
February 2016: Indian Journal of Pediatrics
Heinrike Schmeling, Michael Mahler, Deborah M Levy, Katharine Moore, Anne M Stevens, James Wick, Jacob D McMillan, Gerd Horneff, Shervin Assassi, Julio Charles, Gloria Salazar, Maureen D Mayes, Earl D Silverman, Marissa Klien-Gitelman, Tzelan Lee, Hermine I Brunner, Ann M Reed, Marvin J Fritzler
OBJECTIVE: Autoantibodies to the dense fine speckled 70 kDa antigen (DFS70) are reported to be more common in individuals who do not have an antinuclear antibody (ANA)-associated rheumatic disease (AARD) than in patients with AARD. The frequency of anti-DFS70 antibodies has been thoroughly studied in adult but not in pediatric populations. The primary objective of this observational study was to determine the frequency of anti-DFS70 in pediatric AARD and reference cohorts. METHODS: Sera from 743 children with AARD and related conditions, and 345 samples from reference cohorts (healthy children and those being investigated for AARD) were studied for anti-DFS70 autoantibodies as measured by a chemiluminescence immunoassay...
December 2015: Journal of Rheumatology
Cyril Gitiaux, Marie De Antonio, Jessie Aouizerate, Romain K Gherardi, Thomas Guilbert, Christine Barnerias, Christine Bodemer, Karine Brochard-Payet, Pierre Quartier, Lucile Musset, Bénédicte Chazaud, Isabelle Desguerre, Brigitte Bader-Meunier
OBJECTIVE: Outcome of JDM is highly heterogeneous. Our objective was to determine clinical and muscle biopsy features associated with poor outcome and response to treatment. METHODS: Clinical data and muscle biopsy were obtained from a monocentric cohort of 29 patients. Clinical subgroups were defined by latent class model analysis of initial and follow-up parameters. Myopathological features were analysed using validated scores. Capillary loss was determined on reconstructions of transversal sections and assessed in the different age groups to take into account variations of muscle capillarization during post-natal development...
March 2016: Rheumatology
Emine Ayça Cimbek, Yaşar Şen, Sevil Arı Yuca, Demet Çam, Celal Gür, Harun Peru
Association of Kocher-Debré-Semelaigne syndrome-a myopathy of hypothyroidism in childhood characterized by muscular hypertrophy, with rhabdomyolysis is very rare. We present a case of Kocher-Debré-Semelaigne syndrome with rhabdomyolysis secondary to Hashimoto's thyroiditis. He had muscular symptoms simulating poly/dermatomyositis, massively elevated creatine kinase (CK) levels and high creatinine levels. All of the findings reversed on treatment of hypothyroidism. The response to the therapy strongly suggested that Kocher-Debré-Semelaigne (KDS) syndrome was the underlying etiology...
November 1, 2015: Journal of Pediatric Endocrinology & Metabolism: JPEM
Beverley Almeida, Raquel Campanilho-Marques, Katie Arnold, Clarissa A Pilkington, Lucy R Wedderburn, Kiran Nistala
OBJECTIVE: The Pediatric Rheumatology International Trials Organisation (PRINTO) recently published criteria for classification of patients with juvenile dermatomyositis (DM) as having clinically inactive disease. The criteria require that at least 3 of 4 conditions be met, i.e., creatine kinase level ≤150 units/liter, Childhood Myositis Assessment Scale score ≥48, Manual Muscle Testing in 8 muscles score ≥78, and physician's global assessment of overall disease activity (PGA) ≤0...
September 2015: Arthritis & Rheumatology
Gisele M C Fabri, Cynthia Savioli, José T Siqueira, Lucia M Campos, Eloisa Bonfá, Clovis A Silva
Gingivitis and periodontitis are immunoinflammatory periodontal diseases characterized by chronic localized infections usually associated with insidious inflammation This narrative review discusses periodontal diseases and mechanisms influencing the immune response and autoimmunity in pediatric rheumatic diseases (PRD), particularly juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (C-SLE) and juvenile dermatomyositis (JDM). Gingivitis was more frequently observed in these diseases compared to health controls, whereas periodontitis was a rare finding...
July 2014: Revista Brasileira de Reumatologia
G Esther A Habers, Marco Van Brussel, Kavish J Bhansing, Esther P Hoppenreijs, Anjo J W M Janssen, Annet Van Royen-Kerkhof, Sigrid Pillen
INTRODUCTION: We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM). METHODS: Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT) and quantitative muscle echo intensity (EI) were assessed with QMUS in 4 muscles. RESULTS: Six patients experienced a monocyclic course. At diagnosis EI was slightly increased, and MT was relatively normal...
October 2015: Muscle & Nerve
Alessandro Consolaro, Giulia C Varnier, Alberto Martini, Angelo Ravelli
The search for biomarkers in paediatric rheumatic diseases, particularly juvenile idiopathic arthritis (JIA), childhood lupus nephritis (LN), and juvenile idiopathic inflammatory myopathies (JIIMs) is attracting increased interest. In JIA, a number of biomarkers have shown potential for predicting clinical phenotype, disease activity and severity, clinical remission and relapse, response to treatment, and disease course over time. In systemic JIA, measurement of biomarkers that reflect the degree of activation and expansion of T cells and macrophages might be helpful for detecting subclinical macrophage activation syndrome...
May 2015: Nature Reviews. Rheumatology
Floranne C Ernste, Ann M Reed
PURPOSE OF REVIEW: Ongoing research continues to advance our understanding of the juvenile idiopathic inflammatory myopathies (JIIMs). We review the recent contributions from the published literature about the classification, pathogenesis, assessment, and treatment of JIIMs in basic and translational science and clinical research in 2013 through early 2014. RECENT FINDINGS: Large registries, such as the Childhood Arthritis and Rheumatology Research Alliance registry, are conducting trials to enhance our understanding of JIIMs...
November 2014: Current Opinion in Rheumatology
T C M Castro, H Lederman, M T Terreri, W I Caldana, E Zanoteli, M O E Hilário
OBJECTIVES: Our aim was to demonstrate the benefit of whole-body magnetic resonance imaging (WBMRI) as a diagnostic modality in the detection of muscle activity in juvenile dermatomyositis (JDM)/polymyositis (JPM) patients and to correlate these findings with clinical evaluation, laboratory examinations, nailfold capillaroscopy (NFC), and muscle biopsy. METHOD: Thirty-four patients aged 5.5 to 18.9 years with a diagnosis of JDM/JPM were prospectively evaluated using clinical examination, muscle enzyme determination, the Childhood Myositis Assessment Scale (CMAS), Disease Activity Score (DAS), Manual Muscle Testing (MMT), NFC, and WBMRI...
2014: Scandinavian Journal of Rheumatology
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