Intravenous immunoglobulins as first-line treatment in idiopathic inflammatory myopathies: A pilot study

Johan Lim, Filip Eftimov, Camiel Verhamme, Esther Brusse, Jessica E. Hoogendijk, Christiaan G.J. Saris, Joost Raaphorst, Rob J. De Haan, Ivo N. Van Schaik, Eleonora Aronica, Marianne De Visser, Anneke J. Van Der Kooi*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

28 Citations (Scopus)
33 Downloads (Pure)


Objectives: We explored efficacy and safety of IVIg as first-line treatment in patients with an idiopathic inflammatory myopathy. Methods: In this investigator-initiated phase 2 open-label study, we included 20 adults with a newly diagnosed, biopsy-proven idiopathic inflammatory myopathy, and a disease duration of less than 9 months. Patients with IBM and prior use of immunosuppressants were excluded. The standard treatment regimen consisted of IVIg (Privigen) monotherapy for 9 weeks: a loading dose (2 g/kg body weight) and two subsequent maintenance doses (1 g/kg body weight) with a 3-week interval. The primary outcome was the number of patients with at least moderate improvement on the 2016 ACR/EULAR Total Improvement Score. Secondary outcomes included time to improvement, the number of patients requiring rescue medication and serious adverse events. Results: We included patients with DM (n = 9), immune-mediated necrotizing myopathy (n = 6), non-specific myositis/overlap myositis (n = 4) and anti-synthetase syndrome (n = 1). One patient was excluded from analyses because of minimal weakness resulting in a ceiling effect. Eight patients (8/19 = 42.0%; Clopper-Pearson 95% CI: 19.6, 64.6) had at least moderate improvement by 9 weeks. Of these, six reached improvement by 3 weeks. Seven patients required rescue medication due to insufficient efficacy and prematurely ended the study. Three serious adverse events occurred, of which one was pulmonary embolism. Conclusion: First-line IVIg monotherapy led to at least moderate improvement in nearly half of patients with a fast clinical response in the majority of responders. Trial registration: Netherlands Trial Register identifier, NTR6160.

Original languageEnglish
Pages (from-to)1784-1792
Number of pages9
JournalRheumatology (Bulgaria)
Issue number4
Early online date25 Oct 2020
Publication statusPublished - 1 Apr 2021

Bibliographical note

Publisher Copyright:
© 2020 The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology.


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