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Malignancy in dermatomyositis and polymyositis

INTRODUCTION

Dermatomyositis (DM) and polymyositis (PM) are two distinct forms of inflammatory myopathy. The hallmark of these disorders is muscle weakness. However, a variety of other disease manifestations are also characteristic of these conditions, even though not all features are found in every patient. These include:

  • Skin findings such as Gottron's sign and heliotrope rash (DM only)
  • Interstitial lung disease
  • Raynaud phenomenon
  • Inflammatory arthritis
  • Serum autoantibodies

In addition, both DM and PM are associated with malignancies in a significant minority of cases. The close relationship between inflammatory myopathy and cancer is consistent with the concept that paraneoplastic processes linked to oncogenesis and autoimmunity contribute to the disease in a subset of DM and PM cases.

Issues related to malignancy in patients with DM and PM will be reviewed here. The clinical manifestations, diagnosis, and management of both the adult and juvenile forms of these disorders are discussed separately. (See "Clinical manifestations and diagnosis of adult dermatomyositis and polymyositis" and "Initial treatment of dermatomyositis and polymyositis in adults" and "Pathogenesis and clinical manifestations of juvenile dermatomyositis and polymyositis" and "Treatment of juvenile dermatomyositis and polymyositis".)

PATHOPHYSIOLOGY

The precise links between malignancy and inflammatory myopathy remain incompletely understood [1,2]. However, the temporal relationship between the development of cancer and diagnosis of inflammatory myopathy is reminiscent of that seen in neurologic paraneoplastic disorders in which the antigens targeted for an immune response are expressed at high levels in both the inciting tumor and the affected neuronal tissue [3,4]. (See "Overview of paraneoplastic syndromes of the nervous system".)
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