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Review
. 2025 Sep 24;24(10):103879.
doi: 10.1016/j.autrev.2025.103879. Epub 2025 Jul 14.

Remission and low disease activity definitions in adult idiopathic inflammatory myopathies: A narrative review by myositis clinical trials consortium (MCTC)

Nantakarn Pongtarakulpanit  1 , Shiri Keret  2 , Vaidehi Kothari  3 , Francisca Bozán  4 , Chengappa Kavadichanda  5 , Akira Yoshida  6 , Valérie Leclair  7 , Anuradha Bishnoi  8 , Kaveh Ardalan  9 , Edoardo Conticini  10 , Ting-Yuan Lan  11 , Océane Landon-Cardinal  12 , Iris Y K Tang  13 , Silvia Rosina  14 , Belina Y Yi  15 , James B Lilleker  16 , Eduardo Dourado  17 , Prateek C Gandiga  18 , Rohit Aggarwal  19
Affiliations
Review

Remission and low disease activity definitions in adult idiopathic inflammatory myopathies: A narrative review by myositis clinical trials consortium (MCTC)

Nantakarn Pongtarakulpanit et al. Autoimmun Rev. .

Abstract

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of rare systemic autoimmune rheumatic diseases. Despite advances in treatment, the definition of remission and low disease activity (LDA) in IIM remains inconsistent and lacks consensus and validation. This review summarizes existing published definitions, achievement rates, and predictive factors of remission/LDA in adult IIM, focusing on dermatomyositis (DM), polymyositis (PM), anti-synthetase syndrome (ASyS), and immune-mediated necrotizing myopathies (IMNM). Our literature review revealed a wide variability in remission definitions, incorporating physician assessment, muscle strength, laboratory normalization, and medication tapering or discontinuation. Some studies defined "remission on medication", while others required complete treatment cessation. Most definitions required a minimum duration of six months. Organ-specific remission (including for the skin, lung, and muscle domains) was inconsistently addressed. LDA has been less extensively studied in IIM, with the myositis disease activity assessment visual analog scales (MYOACT) being the only measure applied to DM. Remission rates varied widely, with stricter criteria yielding lower rates. Factors associated with remission included younger age, early immunosuppressive treatment, non-severe muscle involvement, the absence of myositis-specific autoantibodies (MSA), although some studies reported positivity for certain MSA were associated with remission. Conversely, remission was less likely for patients with PM, overlap myositis, and those positive for anti-TIF1-γ or Ku autoantibodies. Standardized remission criteria incorporating physician assessment, patient assessment, organ-specific parameters, laboratory assessments, and sustained remission duration are essential for harmonizing clinical and research evaluations in IIM. Establishing uniform definitions will improve therapeutic outcome assessments and facilitate meaningful comparisons in clinical trials and real-world practice.

Keywords: Anti-synthetase syndrome; Dermatomyositis; Disease activity; Myositis; Polymyositis; Predictor; Remission.

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Conflict of interest statement

Declaration of competing interest RA has received grants/research fundings from Mallinckrodt, Pfizer Inc., Bristol Myers-Squibb, Q32, EMD Serono, Janssen, and Boehringer Ingelheim; and has served as a consultant for Octapharma, CSL Behring, Bristol-Myers Squibb, EMD Serono, Kezar, Pfizer Inc., AstraZeneca, Alexion, Argenx, Corbus, Janssen, Kyverna, Roivant, Merck, Galapagos, ActiGraph, Horizon Therapeutics, Teva, ANI Pharmaceutical, Nuvig, Capella, Cabaletta Bio, I-Cell, Sanofi, and Boehringer Ingelheim. PG has no direct conflicts of interest with this work. He has served as PI/co-PI and/or safety officer/data monitoring board member for multiple sponsored clinical trials in IIM. He has received honoraria for advisory board/monitoring board services from Kezar Life Sciences, Jannsen Pharmaceuticals, and the National Institutes of Health. BY has received non-financial support from the Childhood Arthritis and Rheumatology Research Alliance (administrative assistance). KA has served on a scientific advisory board and performed paid consulting for Cabaletta Bio; servies as Vice Chair of the Childhood Arthritis & Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Committee (paid consultant position); received travel reimbursement and honoraria from the Rheumatology Research Foundation (Pediatric Visiting Professorship 2024–2025); received travel reimbursement from the American College of Rheumatology; performed grant pre-review for SingHealth (honorarium paid directly to institution, not to KA); received research funding from the Rheumatology Research Foundation, CARRA/Arthritis Foundation, Chan Zuckerberg Initiative, Cure JM Foundation, Patient-Centered Outcomes Research Institute, and National Center for Advancing Translational Sciences/Technical Resources International. The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Rohit Aggarwal reports a relationship with Mallinckrodt, Pfizer Inc., Bristol Myers-Squibb, Q32, EMD Serono, Janssen, and Boehringer Ingelheim that includes: funding grants. Rohit Aggarwal reports a relationship with Octapharma, CSL Behring, Bristol-Myers Squibb, EMD Serono, Kezar, Pfizer Inc., AstraZeneca, Alexion, Argenx, Corbus, Janssen, Kyverna, Roivant, Merck, Galapagos, ActiGraph, Horizon Therapeutics, Teva that includes: consulting or advisory. Rohit Aggarwal reports a relationship with ANI Pharmaceutical, Nuvig, Capella, Cabaletta Bio, I-Cell, Sanofi, and Boehringer Ingelheim that includes: consulting or advisory. Prateek C. Gandiga reports a relationship with Kezar Life Sciences, Jannsen Pharmaceuticals, and the National Institutes of Health that includes: consulting or advisory. Belina Y. Yi reports a relationship with CARRA Inc. that includes: non-financial support. Kaveh Ardalan reports a relationship with Cabaletta Bio that includes: consulting or advisory. Kaveh Ardalan reports a relationship with CARRA Inc. that includes: consulting or advisory and funding grants. Kaveh Ardalan reports a relationship with the Rheumatology Research Foundation that includes: funding grants and travel reimbursement. Kaveh Ardalan reports a relationship with American College of Rheumatology that includes: travel reimbursement. Kaveh Ardalan reports a relationship with SingHealth Group that includes: consulting or advisory. Kaveh Ardalan reports a relationship with Chan Zuckerberg Initiative, Cure JM Foundation, Patient-Centered Outcomes Research Institute, and National Center for Advancing Translational Sciences that includes: funding grants. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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