Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Dermatomyositis (DM) and polymyositis (PM) are rare chronic inflammatory disorders of muscle. The morbidity and mortality associated with these conditions remains significant despite treatment, which typically begins with high-dose corticosteroids. Second-line agents are commonly used in clinical practice, however there are no clear evidence-based guidelines directing their use. We systematically assessed the evidence for immunotherapy in DM and PM.
Methods: Relevant studies were identified through Ovid Medline and PubMed database searches. Bibliographies of relevant studies were scrutinized for other potentially relevant citations, and research registers of ongoing trials and international conference proceedings were examined to identify research in progress or data as yet unpublished. Randomized controlled trials and experimental studies without true randomization (quasi-randomized) including adult patients with definite or probable DM or PM were evaluated. Trials involving patients with possible, early or mild disease were excluded, as were those where diagnostic certainty was unknown or diagnostic criteria had not been specified. Any type of immunotherapy was considered. Improvement in muscle strength was the primary outcome. Secondary outcomes included improvements in patient and physician global scores, physical function and muscle enzymes. Studies not assessing these outcomes were excluded. Using predetermined criteria, the two authors independently selected trials for inclusion and then assessed these for quality.
Results: 1697 citations were retrieved. 13 trials were identified as potentially relevant, 3 were excluded after full text review. 2 further trials were identified after hand-searching reference lists. 12 studies were selected for full analysis, including a total of 522 participants. Differences in trial design and quality, and variable reporting of baseline characteristics and outcomes made direct comparison impossible. Although no one treatment can be recommended on the basis of this review, improved outcomes were demonstrated with a number of agents including methotrexate, azathioprine, ciclosporin and intravenous immunoglobulin. Plasmapheresis and leukapheresis were of no benefit. Biologics were well tolerated but were not demonstrated to be of benefit.
Study |
Intervention |
Conclusion |
Bunch et al 1980 |
Pred + AZA vs. Pred + Placebo |
No additional benefit with AZA at 3 months |
Hollingworth et al 1982 |
ALG + AZA vs. Pred |
NS trend toward benefit with ALG + AZA |
Miller et al 1992 |
PEX vs. Leukapheresis vs. Sham apheresis |
PEX/Leukapheresis of no benefit |
Dalakas et al 1993 |
IVIg vs. Placebo |
IVIg beneficial in refractory DM |
Villalba et al 1998 |
MTX + AZA + Pred vs. IV MTX + Pred |
NS trend toward benefit with MTX + AZA |
Vencovsky et al 2000 |
CsA + Pred vs. MTX + Pred |
MTX and CsA equivalent, but MTX better tolerated |
Coyle et al 2008 |
IFX vs. Placebo |
IFX is well tolerated, but has limited efficacy |
Van de Vlekkert et al 2010 |
Pred vs. Dex |
Dex not superior to Pred, but fewer side effects |
The Muscle Study Group 2011 |
Pred +ETAN vs. Pred + Placebo |
No additional benefit with ETAN, but has steroid-sparing effect |
Miyasaka et al 2011 |
IVIg vs. Placebo |
IVIg of no benefit in corticosteroid-refractory PM/DM |
Choy et al 2011 |
Pred vs. Pred + MTX vs. Pred + CsA vs. Pred + MTX + CsA |
Adding immunosuppressants to corticosteroid therapy offers no additional benefit |
Oddis et al 2013 |
Ritux early vs. Ritux late |
No significant difference between groups, but 83% met DOI |
ALG, anti-lymphocyte globulin; AZA, azathioprine; CsA, ciclosporin; Dex, dexamethasone; ETAN, etanercept; IFX, infliximab; IV, intravenous; IVIg, intravenous immunoglobulin; MTX, methotrexate; NS, non-significant; PEX, plasma exchange; Pred, prednisolone; Ritux, rituximab. |
Conclusion: More high quality randomized controlled trials are needed to establish the role of second-line agents, in particular biologics, in the treatment of DM and PM.
Disclosure:
E. Vermaak,
None;
N. J. McHugh,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/evidence-for-immunotherapy-in-polymyositis-and-dermatomyositis-a-systematic-review/