Long-term oral glucocorticoid use is associated with complications, healthcare resource utilization, and costs among patients with dermatomyositis or polymyositis.

Introduction/objective: Oral glucocorticoids (OGC) are conventionally used as first-line treatment for dermatomyositis (DM) and polymyositis (PM). This study evaluated clinical and economic outcomes associated with long-term (LT) OGC use in DM/PM.

Methods: Adults with ≥ 2 medical claims of DM/PM 30‒365 days apart from January 1, 2016, to December 31, 2022, and ≥ 1 diagnosis code of a physician specialty of interest were selected from the MarketScan Commercial and Medicare Supplemental databases.

A Scoping Review of Respiratory Dysfunction in Inclusion Body Myositis.

Objectives: Inclusion body myositis (IBM) can result in deadly respiratory consequences. Yet, the mechanism driving this issue remains equivocal. We mapped the literature to identify physiological respiratory characteristics in IBM and the types of respiratory assessments used.

Item selection for the development and validation of a revised classification criteria for adult and juvenile idiopathic inflammatory myopathies: MyoROC project.

Objective: A revision of the 2017 EULAR-ACR myositis classification criteria, namely EULAR-ACR funded Myositis Revision of Classification (MyoROC) project, is currently underway involving a large international group of experts. In the first phase of this project, we identified additional items to be tested in the criteria.

Methods: We distributed an electronic survey to International Myositis Assessment and Clinical Studies (IMACS) members to identify new items.

Performance of sit-to-stand, timed up-and-go, and six-minute walk tests in home and office settings in patients with idiopathic inflammatory myopathy.

Background: Patients with idiopathic inflammatory myopathies (IIM) frequently experience limitations in their physical function. Sit-to-stand (STS), timed-up-and-go (TUG), and six-minute walk distance (6MWD) are task-oriented functional tests that can provide objective information about physical function. In this study, we assess their measurement properties in office and home settings.

Anti-Mi2 autoantibodies target PHD fingers of SP140L and TIF1γ, while anti-TIF1γ autoantibodies primarily bind TIF1γ outside the PHD region.

Objectives: Plant homeodomain (PHD) fingers are present in many chromatin-binding proteins. We recently discovered that anti-Mi2 autoantibodies recognize PHD fingers in Mi2 and AIRE. The purpose of this study was to characterize anti-Mi2 autoantibody recognition of PHD fingers in SP140L and TIF1γ as well as to explore recognition of TIF1γ by both anti-TIF1γ and anti-Mi2 autoantibodies.

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

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).

Efficacy of Intravenous Immunoglobulin for Systemic Manifestations of Dermatomyositis Beyond Muscular and Cutaneous: Sub-analysis of the ProDERM Study.

Introduction: Muscle and skin involvement are well defined in dermatomyositis but other symptoms contribute significantly to the disease burden and their treatment is not well characterized. This post hoc analysis of ProDERM assessed the effect of intravenous immunoglobulin (IVIg) treatment on other manifestations of dermatomyositis beyond muscular and cutaneous involvement.

Methods: ProDERM was a randomized, placebo-controlled study.

Long-term clinical prognosis of anti-aminoacyl-tRNA synthetase antibodies and interstitial lung disease.

Introduction: Anti-aminoacyl tRNA synthetase (anti-ARS) antibody is the most common myositis-specific antibody subtype. Anti-ARS antibody-positive myositis is often complicated by interstitial lung disease (ILD), but the clinical progression of anti-ARS antibody-positive ILD (ARS-ILD) remains unclear.

Method: A prospectively collected, single center longitudinal myositis database was used to retrospectively investigate 131 patients with ARS-ILD based on subtypes of anti-ARS antibodies (Jo-1, PL-7, PL-12, EJ, OJ, and KS).

Anti-Mi2 autoantibodies target the PHD fingers of SP140L and TIF1γ, while anti-TIF1γ autoantibodies primarily recognize epitopes outside the PHD region of TIF1γ.

Objectives: Plant homeodomain (PHD) fingers are present in many chromatin-binding proteins. We recently discovered that anti-Mi2 autoantibodies recognize PHD fingers in Mi2 and AIRE. The purpose of this study was to characterize anti-Mi2 autoantibody recognition of PHD fingers in SP140L and TIF1γ as well as to explore recognition of TIF1γ by both anti-TIF1γ and anti-Mi2 autoantibodies.

Consensus nomenclature and abbreviation for anti-synthetase syndrome: an IMACS project.

Objectives: The lack of uniform terminology and abbreviations for anti-synthetase syndrome has led to significant challenges in research and clinical practice. This study aimed to establish an international consensus on a standardized nomenclature and abbreviation among a diverse group of global myositis experts and patient representatives.

Methods: This qualitative project was approved by the International Myositis Assessment and Clinical Studies Group (IMACS).

Short Form-36 psychometric properties in idiopathic inflammatory myopathies: reliability, validity and responsiveness.

Objective: To evaluate the psychometric properties of Short Form-36 (SF-36) as a health-related quality of life (HRQoL) measure in idiopathic inflammatory myopathies (IIMs).

Methods: Patients fulfilling the EULAR/ACR classification criteria or with myositis-specific antibodies were enrolled from two clinical trials (Tocilizumab in Myositis and Abatacept in Myositis) and one prospective observational study. Data collection at 0, 3 and 6 months included all myositis core set measures (CSMs) including manual muscle testing-8 (MMT-8), and SF-36 summary scores and all eight subdomains including physical component summary score (PCS) and physical function.

Fatigue is common in myositis and is associated with disease activity.

Background: Fatigue is a prevalent and debilitating symptom frequently reported by patients with idiopathic inflammatory myopathies (IIM). This study investigated the reliability, validity and responsiveness of fatigue in myositis, along with its correlation with disease activity.

Methods: Adults with IIM were enrolled in a prospective observational study.

Abatacept for the treatment of myositis-associated interstitial lung disease (ATtackMy-ILD).

Objectives: This randomized, placebo-controlled pilot trial evaluated the efficacy and safety of abatacept in patients with anti-synthetase syndrome-associated interstitial lung disease (ASyS-ILD).

Methods: Participants with active ASyS-ILD were randomized to receive abatacept (n = 9) or placebo (n = 11) for 24 weeks, followed by a 24-week open-label extension with abatacept for all participants. The primary end point was a change in % predicted forced vital capacity (%FVC) from baseline to week 24.

Recruitment rate comparison between a virtual tele-research cohort and a traditional centre-based cohort in idiopathic inflammatory myopathy.

Objectives: Idiopathic inflammatory myopathy (IIM, myositis) faces recruitment and retention challenges in clinical studies due to disease rarity, geographical barriers and logistic difficulties. This study aimed to compare recruitment, enrolment, retention and data completion between a decentralized telemedicine-based research cohort (TRC) and a traditional centre-based cohort (CBC) in IIM.

Methods: The Myositis Patient Centered Research (MyPacer) study, a large prospective observational study, collected data on core set measures, patient-reported outcomes and functional tests.

The role of multicriteria decision analysis in the development of candidate classification criteria for antisynthetase syndrome: analysis from the CLASS project.

Objectives: To develop and evaluate the performance of multicriteria decision analysis (MCDA)-driven candidate classification criteria for antisynthetase syndrome (ASSD).

Methods: A list of variables associated with ASSD was developed using a systematic literature review and then refined into an ASSD key domains and variables list by myositis and interstitial lung disease (ILD) experts. This list was used to create preferences surveys in which experts were presented with pairwise comparisons of clinical vignettes and asked to select the case that was more likely to represent ASSD.

Role of CD19 Chimeric Antigen Receptor T Cell Therapy in Idiopathic Inflammatory Myopathies.

Idiopathic inflammatory myopathies (IIMs) comprise a spectrum of autoinflammatory disease characterized primarily by muscle inflammation, with secondary involvement of diverse organs including joints, skin, lungs, heart, and the gastrointestinal system. Managing these conditions poses considerable challenges, often inflicting profound distress on the afflicted individuals. Encouragingly, the deployment of chimeric antigen receptor (CAR) T cell therapy has demonstrated promising efficacy across various autoimmune diseases, extending hope for ameliorating the burden of IIM.

The Myositis Clinical Trials Consortium: an international collaborative initiative to promote clinical trials in adult and juvenile myositis.

Idiopathic inflammatory myopathies (IIM), or myositis, are a heterogeneous group of systemic autoimmune disorders that are associated with significant morbidity and mortality. Conducting high-quality clinical trials in IIM is challenging due to the rare and variable presentations of disease. To address this challenge, the Myositis Clinical Trials Consortium (MCTC) was formed.

Predictors of response to intravenous immunoglobulin in patients with dermatomyositis: the ProDERM study.

Objectives: The phase 3 ProDERM study demonstrated intravenous immunoglobulin (IVIg) was safe and effective in patients with dermatomyositis (DM). This analysis assessed clinical and serological predictors of IVIg response in DM patients from ProDERM.

Methods: ProDERM was a prospective, randomized, placebo-controlled study of DM patients.

Efficacy, safety, and target engagement of dazukibart, an IFNβ specific monoclonal antibody, in adults with dermatomyositis: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.

Background: Dermatomyositis is a chronic autoimmune disease with distinctive cutaneous eruptions and muscle weakness, and the pathophysiology is characterised by type I interferon (IFN) dysregulation. This study aims to assess the efficacy, safety, and target engagement of dazukibart, a potent, selective, humanised IgG1 neutralising monoclonal antibody directed against IFNβ, in adults with moderate-to-severe dermatomyositis.

Methods: This multicentre, double-blind, randomised, placebo-controlled, phase 2 trial was conducted at 25 university-based hospitals and outpatient sites in Germany, Hungary, Poland, Spain, and the USA.

Efficacy and Safety of Subcutaneous Abatacept Plus Standard Treatment for Active Idiopathic Inflammatory Myopathy: Phase 3 Randomized Controlled Trial.

Objective: Our objective was to evaluate the efficacy and safety of subcutaneous (SC) abatacept and standard of care (SOC) for the treatment of idiopathic inflammatory myopathy (IIM) over 52 weeks.

Methods: In this randomized, double-blind, placebo-controlled phase III trial, patients with treatment-refractory IIM received SC abatacept (at 125 mg weekly) with SOC (abatacept group) or a placebo with SOC (placebo group). A 24-week double-blind period was followed by an open-label period to assess outcomes from continued therapy with abatacept and initiation with abatacept (placebo-to-abatacept switch group) from 24 to 52 weeks.

Relationship between high-resolution computed tomography quantitative imaging analysis and physiological and clinical features in antisynthetase syndrome-related interstitial lung disease.

Objectives: To explore the association between the extent of CT abnormalities by quantitative imaging analysis (QIA) and clinical/physiological disease parameters in patients with antisynthetase syndrome associated interstitial lung disease (ARS-ILD).

Methods: We analysed 20 patients with antisynthetase antibodies and active ILD enrolled in the Abatacept in Myositis-Associated Interstitial Lung Disease study. High-resolution chest CT was obtained at weeks 0, 24 and 48 and QIA scored the extent of ground glass (quantitative score for ground glass), fibrosis (quantitative score for lung fibrosis, QLF) and total ILD (quantitative ILD, QILD).

Design of a randomised controlled hybrid trial of nintedanib in patients with progressive myositis-associated interstitial lung disease.

Background: The Myositis Interstitial Lung Disease Nintedanib Trial (MINT) is a hybrid trial, which is enrolling patients both at local sites and remotely via a decentralised site. The trial will investigate the efficacy and safety of nintedanib in patients with progressive myositis-associated interstitial lung disease (MA-ILD).

Methods/design: MINT is an exploratory, prospective randomised placebo-controlled trial.