الفهرس 
Definition and ClassificationOnly 14 pages are availabe for public view
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Abstract
uvenile dermatomyositis is the most common of the idiopathic inflammatory myopathies in children. It is considered an autoimmune disease of relatively unknown etiology, although environmental exposures and infectious agents are thought to play a role in disease pathogenesis. More recently, data has become available regarding the molecular genetics of children affected with juvenile dermatomyositis and the impact these genes have on disease expression and clinical course.
Over the past 10 to 15 years, much has been learned about the cellular and humoral immune-mediated mechanisms involved in the pathogenesis of JDM. More recently, the role of complement, specifically the membrane attack complex, in immune-mediated vascular injury has been further elucidated. Additionally, susceptibility to developing JDM has been linked with the class II major histocompatibility complex HLA-DQA1*0501 allele, and disease course and various complications have been associated with polymorphisms at the TNFα-308 locus.
Juvenile dermatomyositis remains a great challenge for all professional involved in its management. Early diagnosis and the fast institution of adequate therapy are fundamental determinants to achieve good results and quality of life for the affected child.
The optimal recognition, detailed full assessment, and treatment, of children with JDM requires specialist multidisciplinary teams, working in centres with experience with this group of rare and serious conditions.
New insights into pathogenesis, myositis-associated autoantibodies and genetic risk factors are generating novel ways to assess patients with JDM, and are likely to provide predictive biomarkers that may aid in the diagnosis or ongoing assessment of disease activity.
Standardised approaches to assess JDM disease activity include a combination of measures to assess overall disease activity and damage, muscle weakness, functional disability, laboratory findings and extra-muscular activity, including the skin.
Although controlled evidence is frequently lacking for treatment in JDM, a number of studies confirm the value of aggressive treatment, with the aim to induce stable remission early, and thereby reduce steroid use and prevent disability and complications.
New and combined treatment regimens, including new disease modifying agents and biological therapies, are now in use for refractory JDM in many centres such as: Methotrexate, Cyclosporine, Rituxiamab, anti-TNF- , Intravenous immunoglobulins (IVIG) and IV methyprednisolone.