Difference between revisions of "Dermatomyositis"

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==Clinical Features==
 
==Clinical Features==
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[[File:Heliotrope.png|thumb|Heliotrope rash]]
 
===Muscular===
 
===Muscular===
 
*Proximal greater than distal weakness
 
*Proximal greater than distal weakness

Revision as of 15:05, 19 October 2016

Background

  • Inflammatory myopathy
  • Affects proximal muscles > distal
  • Female:Male; 2:1
  • Peak incidence 40-50s

Clinical Features

Heliotrope rash

Muscular

  • Proximal greater than distal weakness
    • Deltoid and hip flexors
    • difficulty standing from a chair
  • Onset over months
  • Mild myalgias

Dermatologic

  • Heliotrope rash on face scalp
  • Gottron's papules (rash on MCP, PIP, and DIP joints)
  • Shawl sign (rash above shoulders)

Other

  • Cardiac (cardiomyopathy)
  • High association with malignancy
  • Interstial lung disease

Differential Diagnosis

  • Drug Induced Myopathies (Statins)

Weakness

Evaluation

  • CK
  • ANA
  • Anti-Jo-1
  • Chem for creatinine
  • Troponin, can involve myocardium
  • ECG
  • Bedside PFTs (FEV1 and FVC)
  • Urinalysis for myoglobin
  • Consider malignancies
  • Signs of systemic inflammation[1]
    • Fever
    • ESR elevated in 50% of patients, does not correlate with disease activity
    • Elevated CRP

Management

Mild disease

  • Prednisone 1mg/kg/day (up to 80mg/day)
    • Taper after 6-8 weeks

Severe disease

  • Respiratory muscle symptoms of CHF
    • Methylprednisolone 1000mg/day x 3 days

Disposition

  • Admit to monitored bed for CHF symptoms, respiratory muscle weakness
  • Admit for dysphagia/aspiration risk
  • Out patient follow up with muscle biopsy for others
  • Very strongly associated with malignancy (~30%), especially:[2]
    • Ovarian
    • Lung
    • Pancreatic
    • Stomach
    • Colorectal
    • Lymphoma
    • Bladder

See Also

References

  1. Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573.
  2. Hill CL et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001 Jan 13;357(9250):96-100.