Idiopathic Inflammatory myopathy (IIM)

Includes:

  • Dermatomyositis
  • Polymyositis

*Does not include Inclusion Body Myositis (IBM)

RecommendationsIVIG is indicated in patients with IIM as adjunctive therapy to corticosteroids and/or a steroid sparing agent in patients with IIM who have failed 1st line therapy or as clinically indicated in the management of severe disease .
 
*IVIG benefit has not been established in IBM.
 
1st line: Corticosteroids and Methotrexate and/or Azathioprine
2nd line: IVIG
3rd line: Cyclosporine or cellcept
Dose/Frequency of AdministrationMaximum dose is 2 g/kg to be given over 2 days initially monthly for 3-6 months and if effective to be continued at decreasing frequency (determine minimum effective dose) over approximately 2 years.
 
Survival of patients with IIM has been shown to be substantially improved in patients given IVIG