Juvenile dermatomyositis
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Abstract
Objective: To analyze the characteristics of juvenile dermatomyositis (JDM). Bibliographic review: JDM is quite rare, but it is the most common idiopathic inflammatory myopathy in pediatric patients. The disease is characterized by proximal muscle weakness, characteristic rashes, and vasculopathy. The incidence of juvenile dermatomyositis is estimated to be around 2 to 4 per million children, with differences between racial and ethnic groups. Its etiology is unknown, but it is suggested JDM is caused by an autoimmune reaction in genetically susceptible individuals, possibly in response to infection or other environmental agents. Myositis-specific antibodies (MSAs) can be helpful in identifying the disease phenotype. Final considerations: Despite its rarity, JDM is an autoimmune disease that has a great impact on quality of life, and may have potentially lethal complications, such as pulmonary and cardiovascular complications. In this sense, early diagnosis and adequate treatment are essential. Its therapy is performed with first-line drugs (glucocorticoids and methotrexate) and second-line drugs (rituximab, mycophenolate mofetil and cyclophosphamide) targeting the clinically inactive disease. Furthermore, Janus kinase inhibitors show promise in the treatment of refractory forms.
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