Treatment of inflammatory myopathies

ABSTRACT

Introduction: The idiopathic inflammatory myopathies (IIM) dermatomyositis (DM) and polymyositis (PM) are chronic diseases affecting the striated muscles with variable involvement of other organs.

Glucocorticoids are considered the cornerstone of treatment, but some patients require adjunctive immunosuppressive agents because of insufficient response to glucocorticoids, flares upon glucocorticoid tapering, or glucocorticoid-related adverse events.

Areas covered: The aim of this article was to review (PubMed search until February 2018) the evidence on established and new therapies derived from randomized controlled trials (RCTs) on adult DM and PM. In addition, key data from open-label trials, case reports, and abstracts were included where data from RCT were lacking.

Expert commentary: Numerous synthetic and biological immunosuppressive agents are currently available to treat the IIM, sometimes in combination. The choice of the specific medication in the individual patient depends upon the disease phenotype and patient’s characteristics. Exercise improves muscle performance without causing disease flares and should be an integral part of the treatment of the IIM. Prompt diagnosis and treatment can lead to better outcome.

KEYWORDS:

• Biological agents
• dermatomyositis
• disease-modifying anti-rheumatic drugs
• glucocorticoids
• myositis
• polymyositis

Declaration of interest

N Pipitone has been a guest speaker at UCB-sponsored meetings (Immunology Summits, Prague, 2012, 2013, and 2014, MACRO Meet the expert at the ACademy of RheumatOlogy, Bologna 13–14 April 2012, GRAPPA Workshop, Milan 29 January 2016 and Rome 30 November 2017) and Alfa-Wassermann sponsored meeting (Rhewind, Bologna, February 2016); has received royalties from Uptodate.com; and is a primary investigator for Italy for the gevokizumab in myositis Servier study (2014), for the sirukumab in GCA GSK study (2016), for the ToReMy AIFA funded (2017) study, and for the FOREUM funded (2018) GCA study. C Salvarani received consulting and investigator fees from Abbvie, Pfizer, MSD, Roche, Celgene, and Novartis. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper is not funded.