Dermatomyositis – challenges in treatment?

Abstract

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.