RESUMO
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.
Assuntos
Calcinose/diagnóstico , Dermatomiosite/diagnóstico , Lipodistrofia/diagnóstico , Síndrome Metabólica/diagnóstico , Atrofia Muscular/diagnóstico , Adulto , Calcinose/patologia , Pré-Escolar , Dermatomiosite/patologia , Progressão da Doença , Feminino , Humanos , Resistência à Insulina , Lipodistrofia/patologia , Síndrome Metabólica/patologia , Atrofia Muscular/patologia , Índice de Gravidade de DoençaRESUMO
Systemic lupus erythematosus (SLE) is a complex autoimmune rheumatic disease with multiple presentations, whose management presents many challenges. Many disease modifying or immunosuppressive drugs have been used with limited success, especially in patients with more severe disease activity. Belimumab is the first drug to be approved specifically for the treatment of SLE in more than 50 years. By blocking the B-cell activating factor, it interferes in B-cell differentiation and survival. Here we consider the results of the clinical trials that led to its approval, as well as the post-hoc analyses, follow-up studies and the current trials.