JUVENILNI DERMATOMIOZITIS

Lana Tambić Bukovac, Marija Perica, Mandica Vidović, Lovro Lamot, Miroslav Harjaček

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.

Juvenilni dermatomiozitis je najčešća bolest iz skupine idopatskih inflamatornih miopatija, koje predstavljaju heterogenu skupinu subakutnih, kroničnih i akutnih bolesti skeletnih mišića. Jedinstvena klinička prezentacija dermatomiozitisa obilježena je karakterističnim kožnim promjenama i progresivnom slabošću mišića. Juvenilni oblik dermatomiozitisa razlikuje se od adultnog, jer je riječ o sistemnoj vaskulopatiji, koja nije povezana s malignim bolestima te se nerijetko preklapa s drugim sistemnim upalnim bolestima dječje dobi. Imunopatologija JDM je kompleksna, no svakim danom se javljaju nove spoznaje koje upotpunjuju naše znanje o bolesti. Liječenje uvijek započinje kortikosteroidima, a nastavlja se imunomodulatorima, te u novije vrijeme biološkim lijekovima. Suvremeno liječenje kao i bolje poznavanje same bolesti, pridonijele su znatnom smanjenu smrtnosti i poboljšanju kvalitete života.