Dermatomyositis – challenges in treatment?

• Autor(a) principal: Antunes, Rui
• Data de Publicação: 2009
• Outros Autores: Azevedo, Marta, Paz, José, Ferreira, Maria do Sameiro
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://revista.spmi.pt/index.php/rpmi/article/view/1421
• topic: dermatomiosite; prednisolona; azatioprina; metotrexato; ciclofosfamida; imunoglobulina; dermatomyositis; prednisolone; methotrexate; cyclophosphamide; azathioprine; immunoglobulin

The major inflammatory myopathies – dermatomyositis, polymyositis and inclusion body myositis – are uncommon and can be difficult to distinguish from many conditions that mimic them clinically. They have a high morbidity and are not infrequently the first sign of an associated malignancy and they may be a part of another connective tissue disease.The treatment of the idiopathic inflammatory myopathies is challenging for a number of reasons – patient heterogeneity, limited clinical trial data and suboptimal assessment tools to quantitate disease activity and damage and reliably distinguish between them. Despite these limitations and challenges, and after confirming the diagnosis, determining the clinical and serologic subgroup of the patient, assessing extramuscular manifestations and defining the relative contribution of disease activity and damage to the patient’s condition, a therapeutic plan should be developed and followed. Although corticosteroids remain the mainstay of an initial treatment plan, combination immunosuppressive regimens and other novel disease-modifying immunologic agents are new additions to the therapeutic arsenal of inflammatory myopathy. Rehabilitative measures and physical therapy interventions are critical elements to include at every stage in the treatment plan.The authors present two cases of Dermatomyositis, illustrate some of the complexities in the presentation and natural history of these disease and the benefits of intensive therapy.