Özet:

Juvenile dermatomyositis is the most common idiopathic inflamatuary myositis in children. It is a rare, chronic, vasculopathic, autoimmune disorder characterized by symmetrical proximal muscle weakness and pathognomonic skin rash. Macrophage activation syndrome (MAS) is a severe and life theratening complication encountered in the patients of chronic rheumatic diseases of childhood. We reported a 12 year old girl presented with MAS due to juvenile dermatomyositis. She had proximal muscle weakness, heliotrope rash, high level of aldolase as spesific muscle enzyme, typical myositis findings on magnetic resonance imaging (MRI). According to the diagnostic criteria of HLH-2004 MAS was considered secondary to juvenile dermatomyositis. Fever, splenomegaly, cytopenia, hypertrigliseridemia, hemophagocytosis was present. The patient was treated with intravenous immunglobulin and metotrexate. After this treatment muscle weakness was improved day by day. On the fifteenth day of this treatment, proximal upper and lower, neck and shoulder motor extremity strength was improved dramatically. We aimed to represent treatment approach for MAS that is secondary to juvenile dermatomyositis and is a rare condition. For a patient with the diagnosis autoimmune disease, MAS should be considered when unexpected situation is encountered during course of the disease and should and treated immediately.

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