Defining interstitial lung disease related to idiopathic inflammatory myopathies: a systematic review protocol of the Myositis Clinical Trial Consortium (MCTC)

BACKGROUND: Idiopathic inflammatory myopathies (IIM) are systemic autoimmune diseases that include dermatomyositis, polymyositis, antisynthetase syndrome, and immune-mediated necrotizing myopathy in both adult and juvenile populations. Interstitial lung disease (ILD) is a common and serious complication of IIM, with a highly variable clinical course ranging from indolent disease to rapidly progressive respiratory failure, often influenced by underlying myositis-specific autoantibody profiles. Despite the substantial morbidity and mortality associated with IIM-ILD in both adults and children, high-quality evidence to guide clinical management remains limited. A major barrier to progress in this field is the lack of standardized disease nomenclature and uniform diagnostic or classification criteria, which hampers cohort development, limits comparability across studies, and restricts generalizability of findings. To address this gap, we will conduct a systematic review to inform a consensus-based disease definition for adult and juvenile IIM-ILD, with detailed methodology outlined in this protocol. This review will not evaluate prognosis, treatment, or clinical outcomes, but instead focus exclusively on how IIM-ILD is defined and operationalized across studies. METHODS: Following PRISMA 2020 statement and Cochrane methodology, we will search PubMed, EMBASE, Scopus, Cochrane Systematic Reviews, and Cochrane Central from inception to June 30, 2025, using a ''Peer Review of Electronic Search Strategies (PRESS)''-validated strategy. Eligible studies will include randomized controlled trials (RCTs), cohort and case-control studies, systematic reviews, meta-analyses, guidelines, and consensus statements explicitly reporting IIM-ILD definitions in human participants of all ages. Study selection will be performed in Covidence using predefined criteria. Data extraction will capture case definitions, classification criteria, diagnostic methods, ILD patterns, autoantibody profiles, and study characteristics. Quantitative synthesis will summarize the frequency and components of definitions where possible; otherwise, a structured narrative synthesis will be performed. DISCUSSION: Our systematic review protocol is the first comprehensive attempt at synthesizing the highly heterogenous disease nomenclature currently employed in adult and juvenile IIM-ILD literature. Findings will map areas of agreement and inconsistency in existing definitions, forming an evidence base for subsequent consensus processes, such as Delphi, to establish standardized nomenclature. Harmonized definitions are expected to improve research reproducibility, facilitate multicentric collaboration, and improve patient stratification in clinical practice and trials. SYSTEMATIC REVIEW REGISTRATION: PROSPERO ID: 1129967.

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Journal
Systematic Reviews
Published
2026-06-26
DOI
https://doi.org/10.1186/s13643-026-03230-w
Primary Topic
Inflammatory Myopathies and Dermatomyositis
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article
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article

Defining interstitial lung disease related to idiopathic inflammatory myopathies: a systematic review protocol of the Myositis Clinical Trial Consortium (MCTC)

Gwen Wilson, Ramiro Gómez, Robert W. Hallowell, Elena Bartoloni et al.
Systematic Reviews
Inflammatory Myopathies and Dermatomyositis
article

Defining interstitial lung disease related to idiopathic inflammatory myopathies: a systematic review protocol of the Myositis Clinical Trial Consortium (MCTC)

Gwen Wilson, Ramiro Gómez, Robert W. Hallowell, Elena Bartoloni, Pintip Ngamjanyaporn, Kristin Houghton, Shaye Kivity, Charalampia Papadopoulou, Rohit Aggarwal, Víctor R. Pimentel-Quiroz, Jisna Paul, Vasilios Tzilas, Konstantinos Triantafyllias, SUSAN VEHAR, Cristian Alejandro Benítez, Chandana Keshavamurthy, Antonis Fanouriakis, Vivek Nagaraja, Erin M. Wilfong, Elena K. Joerns, Edoardo Marrani, Nikolaos Marketos, Marina Khoury, J. Marsden, Daniel Brito de Araújo, Juan David Urriago, Apostolos Perelas, Océane Landon‐Cardinal, Simone Appenzeller, Komal Ejaz, Clio Mavragani, F M Pais, Ignacio Lopez, Marc Janssen, Solciris A. Dominguez
article en

Abstract

BACKGROUND: Idiopathic inflammatory myopathies (IIM) are systemic autoimmune diseases that include dermatomyositis, polymyositis, antisynthetase syndrome, and immune-mediated necrotizing myopathy in both adult and juvenile populations. Interstitial lung disease (ILD) is a common and serious complication of IIM, with a highly variable clinical course ranging from indolent disease to rapidly progressive respiratory failure, often influenced by underlying myositis-specific autoantibody profiles. Despite the substantial morbidity and mortality associated with IIM-ILD in both adults and children, high-quality evidence to guide clinical management remains limited. A major barrier to progress in this field is the lack of standardized disease nomenclature and uniform diagnostic or classification criteria, which hampers cohort development, limits comparability across studies, and restricts generalizability of findings. To address this gap, we will conduct a systematic review to inform a consensus-based disease definition for adult and juvenile IIM-ILD, with detailed methodology outlined in this protocol. This review will not evaluate prognosis, treatment, or clinical outcomes, but instead focus exclusively on how IIM-ILD is defined and operationalized across studies. METHODS: Following PRISMA 2020 statement and Cochrane methodology, we will search PubMed, EMBASE, Scopus, Cochrane Systematic Reviews, and Cochrane Central from inception to June 30, 2025, using a ''Peer Review of Electronic Search Strategies (PRESS)''-validated strategy. Eligible studies will include randomized controlled trials (RCTs), cohort and case-control studies, systematic reviews, meta-analyses, guidelines, and consensus statements explicitly reporting IIM-ILD definitions in human participants of all ages. Study selection will be performed in Covidence using predefined criteria. Data extraction will capture case definitions, classification criteria, diagnostic methods, ILD patterns, autoantibody profiles, and study characteristics. Quantitative synthesis will summarize the frequency and components of definitions where possible; otherwise, a structured narrative synthesis will be performed. DISCUSSION: Our systematic review protocol is the first comprehensive attempt at synthesizing the highly heterogenous disease nomenclature currently employed in adult and juvenile IIM-ILD literature. Findings will map areas of agreement and inconsistency in existing definitions, forming an evidence base for subsequent consensus processes, such as Delphi, to establish standardized nomenclature. Harmonized definitions are expected to improve research reproducibility, facilitate multicentric collaboration, and improve patient stratification in clinical practice and trials. SYSTEMATIC REVIEW REGISTRATION: PROSPERO ID: 1129967.

Systematic Reviews
Mayo Clinic (US), Harvard University (US), University of Miami (US), Tel Aviv University (IL), Universidade Federal de Pelotas (BR), University of Pittsburgh (US), Universidad de Flores (AR), Universidade Estadual de Campinas (UNICAMP) (BR), Virginia Commonwealth University (US), Johannes Gutenberg University Mainz (DE), National and Kapodistrian University of Athens (GR), Austral University (AR), Universidad Científica del Sur (PE), University of Perugia (IT), Great Ormond Street Hospital for Children NHS Foundation Trust (GB), Meir Medical Center (IL), Banner Health (US), WinnMed (US), The Ohio State University Wexner Medical Center (US), Massachusetts General Hospital (US), University Medical Center of the Johannes Gutenberg University Mainz (DE), Hospital Universitario Austral (AR), Banner - University Medical Center Phoenix (US), Meyer Children's Hospital (IT), Banner - University Medical Center Tucson (US), Mayo Clinic in Arizona (US), Ramathibodi Hospital (TH), University General Hospital Attikon (GR), Hospital Alemán (AR), Centre Hospitalier de l’Université de Montréal (CA), Hospital Posadas (AR), BC Children's Hospital (CA), Hospital Universitario San Ignacio (CO), Henry Dunant Hospital (GR), Piedmont HealthCare (US), Hospital Base Guillermo Almenara Irigoyen (PE), Wilhelmina Children's Hospital (NL), Acura Kliniken (DE), University College London (GB), Vanderbilt University Medical Center (US), Florida College (US)
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Inflammatory Myopathies and Dermatomyositis
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