Dermatomiosite juvenil: revisão e atualização em patogênese e tratamento

Abstract

Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by systemic vasculopathy. Its main manifestations include symmetrical proximal muscle weakness, elevated serum muscle enzymes and cutaneous lesions, among which the heliotrope and Gottron's papules are pathognomonic. Early recognition and prompt therapy allow better prognosis and prevent the development of calcinosis. Although the treatment is based on glucocorticoids, the more commonly associated immunosuppressors include methotrexate, azathioprine, cyclosporine, and cyclophosphamide, depending on the severity of disease. The use of immunobiologicals for refractory cases remains under investigation, but the results are controversial or inexpressive. In this review, we highlight recent updates on the pathogenesis and treatment of JDM.

Adult and juvenile dermatomyositis treatment

“…Treatment options for juvenile dermatomyositis include corticosteroids, intravenous immunoglobulin, hydroxychloroquine, mycophenolate mofetil, azathioprine, cyclophosphamide, cyclosporine, and methotrexate 38–41 …”

“…Other immunosuppressants can be selected based on the severity of the condition. 40,42 Traditional therapies are effective in reducing mortality rates in the long run.…”

“…Treatment options for juvenile dermatomyositis include corticosteroids, intravenous immunoglobulin, hydroxychloroquine, mycophenolate mofetil, azathioprine, cyclophosphamide, cyclosporine, and methotrexate. [38][39][40][41] Among these, high doses of corticosteroids are still the first line of treatment. Other immunosuppressants can be selected based on the severity of the condition.…”

“…9 Early diagnosis and prompt therapy can prevent its development and also result in better outcome. 40 Different therapies including increased systemic immunosuppression, intravenous immunoglobulin, bisphosphonates, and topical sodium thiosulfate are the options, but there is no consensus. 48 Clinical manifestation and myositis-specific antibodies may be useful for prediction of prognosis.…”

Topical action of Buriti oil (Mauritia flexuosa L.) in myositis induced in rats

“…The inflammatory process represents a critical phase of tissue repair, triggered in response to a particular etiological agent. As a defense mechanism, it encompasses a broad contingent of chemical mediators capable of acting at the site of the aggression or systemically 4,5 . The definitive diagnostic procedure for idiopathic inflammatory myopathies (MII) is through muscle biopsy revealing lymphocytic infiltrates, necrosis and regeneration of muscle fibers.…”