RESUMO
Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179-1184.
Assuntos
Autoanticorpos/sangue , Autoantígenos/efeitos dos fármacos , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/imunologia , Imunossupressores/uso terapêutico , Miosite/diagnóstico , Miosite/imunologia , Adolescente , Adulto , Criança , Doença Crônica , Dermatomiosite/diagnóstico , Dermatomiosite/imunologia , Progressão da Doença , Diagnóstico Precoce , Fadiga/imunologia , Humanos , Imunossupressores/administração & dosagem , Debilidade Muscular/imunologia , Miosite de Corpos de Inclusão/diagnóstico , Miosite de Corpos de Inclusão/imunologia , Miosite Orbital/diagnóstico , Miosite Orbital/imunologia , Polimiosite/diagnóstico , Polimiosite/imunologia , Valor Preditivo dos Testes , PrognósticoRESUMO
INTRODUCTION: Juvenile and adult dermatomysitis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. AIM: To compare the symptoms, laboratory and serological findings, treatment and disease course in children and adults suffering from dermatomyositis. METHOD: In this retrospective study, juvenile and adult dermatomyositis groups were formed. There were 27 patients with juvenile dermatomyositis (mean age, 8.7 years; mean follow-up time: 104.6 months) and 30 adult patients (mean age, 50.3; mean follow-up time: 58.1 months). RESULTS: In patients with juvenile dermatomyositis, treatment with intravenous immunoglobulin and cyclosporine A were more frequent as compared to adult patients. Acute onset of the disease was more frequent in adult patients than in those with juvenile disease. In children symptoms of the disease developed gradually. CONCLUSIONS: The findings confirm previously published data showing that there are differences between juvenile and adult patients with dermatomyositis. The authors recommend to follow the patients regularly after reaching remission to avoid bad patient compliance and decrease the number and severity of relapses.
Assuntos
Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Imunossupressores/uso terapêutico , Debilidade Muscular/etiologia , Adolescente , Adulto , Azatioprina/uso terapêutico , Criança , Pré-Escolar , Ciclosporina/uso terapêutico , Dermatomiosite/fisiopatologia , Dermatomiosite/prevenção & controle , Feminino , Seguimentos , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Metotrexato/uso terapêutico , Metilprednisolona/uso terapêutico , Recidiva , Estudos Retrospectivos , Adulto JovemRESUMO
INTRODUCTION: Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by progressive proximal muscle weakness. Cancer-associated myositis represents the worst prognostic group within this heterogeneous disease. AIM: The aim of this study was to reveal factors which increase the risk factors for association of cancerous disease in patients with myositis. Furthermore, the authors explored the most common types of associated malignancies in their patients with myositis and characterize the clinical findings in a sub-group of anti-p155/140 positive patients. METHOD: In this retrospective study, myositis patients with and without associated cancer were analysed (32 and 64 patients, respectively). In addition, anti-p155/140 positive and negative groups were compared, irrespective to the presence of associated malignancies. RESULTS: The risk for associated malignancy was higher in patients with severe muscle and skin symptoms and those with dermatomyositis. Furthermore, increased risk for malignancy was noted in the presence of particular skin symptoms and the absence of systemic symptoms. The anti-p155/140 antibody was proved to be a feasible marker of an independent clinical sub-group which overlapped clinical characteristics with cancer-associated myositis. CONCLUSIONS: These results may help the identification of patients with myositis with a higher risk for associated malignancy.