Professional paper

Juvenile dermatomyositis

Lana Tambić Bukovac ; Children's Hospital Srebrnjak, Zagreb, Croatia
Marija Perica ; Children's Hospital Srebrnjak, Zagreb, Croatia
Mandica Vidović ; Children's Hospital Srebrnjak, Zagreb, Croatia
Lovro Lamot ; Children's Hospital Srebrnjak, Zagreb, Croatia
Miroslav Harjaček ; Children's Hospital Srebrnjak, Zagreb, Croatia

Abstract

Juvenile dermatomyositis is the most common idiopathic inflammatory myopathy in children, and presents a heterogeneous group of subacute, chronic and acute diseases of skeletal muscles. Its unique presentation is marked with characteristic skin rushes and progressive muscle weakness. JDM is clinically distinct from adult dermatomyositis, because it is a systemic vasculopathy not associated with malignancy and it often overlaps with other chronic childhood inflammatory diseases. Although immunopathology of JDM is complex, new studies are completing our knowledge of disease pathogenesis. Corticosteroids represent the first line therapy, afterwards combined with immunomodulatory drugs and biological agents. Better knowledge of the disease combined with modern treatment modalities resulted in reduced mortality rates and in much improved quality of life in patients with JDM.

Keywords

juvenile dermatomyositis; idiopathic inflammatory myopathy; therapy

Hrčak ID:

124044

URI

https://hrcak.srce.hr/124044

Publication date:

25.10.2012.

Article data in other languages: croatian

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