Long-term outcomes in juvenile dermatomyositis: How did we get here and where are we going?

Division of Pediatric Rheumatology, IWK Health Centre, Halifax, Nova Scotia, Canada.
Current Rheumatology Reports (Impact Factor: 2.87). 12/2005; 7(6):441-6. DOI: 10.1007/s11926-005-0048-1
Source: PubMed


Juvenile dermatomyositis (JDM) affects two to four children per million. Prior to treatment with corticosteroids, JDM had a high mortality rate (> 30%) and left 50% of those who survived with serious permanent impairments. After the introduction of corticosteroids, mortality rapidly dropped to less than 10%, and is currently reported to be less than 2% to 3%. Because most children now survive this illness, there is greater interest in long term outcomes. However, review of the literature shows while much is known about mortality, relatively little is known about long term outcomes such as physical function, quality of life, pain, educational and vocational achievement, patient satisfaction, and ongoing disease activity. Furthermore, the literature that has been published has not typically used the same outcomes making comparisons across studies difficult. Current efforts to identify key outcomes and validate measures for those outcomes will allow researchers in the future to provide this much needed information.

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    • "Over the past five decades steroid-based treatment strategies have transformed the prognosis for children with JDM. Before 1960, one-third of patients with JDM would die and one-third developed severe disability [Bitnum et al. 1964; Huber and Feldman, 2005] while the current estimated mortality for JDM is less than 2% [Huber et al. 2000]. "
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    ABSTRACT: Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening 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.
    Full-text · Article · Feb 2012 · Therapeutic advances in musculoskeletal disease
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    ABSTRACT: The clinical profile of 7 children and their follow-up is described. There was female preponderance with mate to female ratio of 1:6. The median age of onset was 6 years. All the patients had skin rash, muscle weakness and abnormal enzyme profile. Muscle biopsy was performed in 6 and was abnormal in all of them. The electromyogram (EMG) was performed in 6 and was found abnormal in five. All the children responded well to corticosteroids. Two children received intravenous dexamethasone bolus and showed good response.
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    ABSTRACT: Recent studies involving juvenile dermatomyositis indicate that the majority of affected children have symptoms suggestive of infection prior to disease onset, damage to skin and muscle each have a distinct pathophysiology, certain urinary muscle metabolites may be useful laboratory markers, and methotrexate used as first line therapy with corticosteroids is associated with greater height velocity and smaller increase in body mass index.
    No preview · Article · Jul 2006 · Current Rheumatology Reports
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