Dermatomyositis/polymyositis

Dermatomyositis/polymyositis is a topic covered in the 5-Minute Emergency Consult.

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Basics

Description

  • Dermatomyositis (DM) and polymyositis (PM) are systemic inflammatory myopathies, which represent the largest group of acquired and potentially treatable causes of skeletal muscle weakness
  • Patients experience a marked progression of muscle weakness over weeks to months
  • Can lead to respiratory insufficiency from respiratory muscle weakness
  • Aspiration pneumonia can occur owing to a weak cough mechanism, pharyngeal muscle dysfunction, and esophageal dysmotility
  • Cardiac manifestations include myocarditis, conduction defects, cardiomyopathy, and congestive heart failure (CHF)
  • Arthralgias of the hands, wrists, knees, and shoulders
  • Ocular muscles are not involved but facial muscle weakness may be seen in advanced cases

Etiology

  • The exact cause is unknown, although autoimmune mechanisms are thought to be largely responsible
  • Incidence ∼1:100,000 with a female preponderance
  • Association with HLA-B8 and HLA-DR3
  • There may be an association between PM and certain viral, bacterial, and parasitic infections
  • DM/PM occurs with collagen vascular disease about 20% of the time
  • In DM, humoral immune mechanisms are implicated, resulting in a microangiopathy and muscle ischemia
  • In PM, a mechanism of T-cell–mediated cytotoxicity is posited. CD8 T cells, along with macrophages surround and destroy healthy, non-necrotic muscle fibers that aberrantly express class I major histocompatibility complex (MHC) molecules
  • Deposition of complement is the earliest and most specific lesion, followed by inflammation, ischemia, microinfarcts, necrosis, and destruction of the muscle fibers

Pediatric Considerations
  • Although DM is seen in both children and adults, PM is rare in children
  • Similar to adult DM, juvenile DM (JDM) primarily affects the skin and skeletal muscles
  • Juvenile form may include vasculitis, ectopic calcifications (calcinosis cutis), and lipodystrophy
  • The juvenile form may be associated with coxsackievirus

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Citation

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TY - ELEC T1 - Dermatomyositis/polymyositis ID - 307704 Y1 - 2016 PB - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307704/all/Dermatomyositis_polymyositis ER -