Juvenile Dermatomyositis Forty Years On: Case Report

Rego de Figueiredo, I; Guerreiro Castro, S; Bernardino, V; Silva Nunes, J; Alves, P; Moraes-Fontes, MF

http://hdl.handle.net/10400.17/3086

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Authors

Rego de Figueiredo, I

Abstract(s)

We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.

Keywords

Calcinosis/diagnosis Calcinosis/pathology Dermatomyositis/diagnosis Dermatomyositis/pathology HCC DAUTOIM HDE IMA HCC END Disease Progression Insulin Resistance Lipodystrophy/diagnosis Lipodystrophy/pathology Metabolic Syndrome/diagnosis Metabolic Syndrome/pathology Muscular Atrophy/diagnosis Muscular Atrophy/pathology Severity of Illness Index