Juvenile dermatomyositis: Advances in understanding and management

The last few decades have produced major advances in the understanding of clinical aspects of inflammatory myositis disorders, both in children and adults. Though relatively rare in childhood, these conditions are often quite severe and require intensive treatments for considerable periods of time. Significant morbidity and occasional mortality still occur despite improvements in outcome. This improved outcome has resulted in part from the novel or better applications of medications already in use, as well as the availability of newer drugs. Few of these have been designed specifically for use in inflammatory myositis but have been drawn from therapeutic trials in other rheumatic disorders and areas of medicine such as oncology and transplantation medicine. Just as important have been the international efforts to design and validate specific outcome measures for all aspects of these disorders, both in childhood and adults, particularly to support the conduct of therapeutic trials. It is highly likely that as improvements in understanding of pathogenesis occur, more specific treatments with drugs such as biological agents will replace the more traditional therapeutic approaches and perhaps designed specifically on a patient-by-patient basis. This review highlights the important advances of the last decade and describes a management approach shared by many of the workers in this field. It focuses primarily on juvenile onset dermatomyositis (JDM) as this is the most common disorder seen, and experience with JDM forms the basis of management of the other disorders.