An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM)

Brown, Vivien; Pilkington, C; Feldman, Brian M.; Davidson, J. A.

doi:10.1093/rheumatology/kel025

Cited by 146 publications

(105 citation statements)

References 5 publications

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“…The diagnosis of JDM is probable if rash is present plus two of the other four criteria are present and definite in the presence of rash and three out of the other four criteria. In an international survey of paediatric rheumatologists published in 2006, clinical manifestations (proximal muscle weakness and characteristic skin rash) and laboratory findings (muscle enzymes) were routinely used in the diagnosis of JDM, while magnetic resonance (MRI) was also deemed important and used by 60% of responders to detect inflammatory muscular changes [34]. Muscle biopsy and EMG were used only by 61.3% and 55.5% [34], respectively, suggesting for the first time that a number of children would fail to meet the current diagnostic criteria necessary for the inclusion in research and other collaborative studies.…”

Section: Diagnosis Of Juvenile Dermatomyositismentioning

confidence: 99%

Treatment of Juvenile Dermatomyositis: An Update

Papadopoulou

Wedderburn

2017

Pediatr Drugs

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Abstract:The Idiopathic Inflammatory Myopathies of childhood consist 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. The advent of biological drugs has revolutionised the management of various paediatric rheumatologic diseases, including inflammatory myopathies. There are few data from randomised controlled trials to guide management decisions, thus several algorithms for the treatment of juvenile myositis have been developed using international expert opinion. The general treatment goals now include elimination of active disease and normalization of physical function, so as to preserve normal growth and development, and to prevent long-term damage and deformities. This review summarizes the newer and possible future therapies of juvenile inflammatory myopathies, including evidence supporting their efficacy and safety.

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Section: Diagnosis Of Juvenile Dermatomyositismentioning

confidence: 99%

Treatment of Juvenile Dermatomyositis: An Update

Papadopoulou

Wedderburn

2017

Pediatr Drugs

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“…Between June 2012 and January 2014, children, adolescents and young adults (ages 2-21 years) with a diagnosis of probable or definite juvenile dermatomyositis (JDM) (as determined by Bohan and Peter [3][4][5] criteria), overlap myositis or polymyositis, were recruited from the pediatric rheumatology clinic and were included regardless of their level of disease activity. After receiving approval from the institutional review board, one of three board-certified pediatric rheumatologists performed a complete evaluation of each patient.…”

Section: Study Populationmentioning

confidence: 99%

“…The diagnosis of JDM is based on the modified criteria of Bohan and Peter [3][4][5]. Although these criteria do not include imaging studies, MRI has been validated as an accurate indicator of muscle edema and active disease [6,7] and is typically incorporated into the diagnostic workup of children with suspected myositis.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

et al. 2015

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Compression-strain US elastography does not accurately detect active myositis in children with juvenile idiopathic inflammatory myopathy and cannot replace MRI as the imaging standard for detecting myositis in these children. The association between abnormal US elastography and increased muscle echogenicity suggests that elastography is capable of detecting muscle derangement in patients with myositis; however further studies are required to determine the clinical significance of these findings.

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“…Although the criteria for diagnosis still used for many studies are still those of Bohan and Peter Peter, 1975a, 1975b] these criteria have been shown to be no longer reflective of Juvenile dermatomyositis: new insights and new treatment strategies modern day paediatric practice, since many paediatricians do not choose to perform electromyography on children, and some centres no longer routinely perform muscle biopsy [Brown et al 2006]. Clinical examination should include formal assessment of muscle strength using a validated tool, such as the Childhood Myositis Assessment Scale (CMAS) or manual muscle testing of the standardized eight groups of muscles (MMT8) [Huber, 2010;Huber et al 2004;Lovell et al 1999].…”

Section: Assessment Of Suspected Cases Of Juvenile Inflammatory Myositismentioning

confidence: 99%

Juvenile dermatomyositis: new insights and new treatment strategies

Martin¹,

Li²,

Wedderburn³

2011

Therapeutic Advances in Musculoskeletal

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Juvenile dermatomyositis (JDM) is a rare but complex and potentially lifethreatening autoimmune disease of childhood, primarily affecting proximal muscles and skin. Although the cause of JDM remains unknown it is clear that genetic and environmental influences play a role in the aetiology. In contrast to adults with dermatomyositis, children with JDM are more likely to have complications that are thought to indicate a vasculopathic process, such as severe skin disease, with ulceration or calcinosis, gut vasculopathy or central nervous system disease. New treatments are much needed and are becoming available and being tested through international multicentre trials. This review will focus on recent insights into pathogenesis, the assessment of the disease in children and the modern approach to its treatment.

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An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM)

Abstract: This process identified nine criteria that clinicians felt to be helpful or important in the diagnosis of JDM. A further process of refinement and validation is necessary to agree an internationally acceptable, clinically usable set of diagnostic criteria.

Cited by 146 publications

References 5 publications

Treatment of Juvenile Dermatomyositis: An Update

Treatment of Juvenile Dermatomyositis: An Update

Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

Juvenile dermatomyositis: new insights and new treatment strategies

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