Shaping the landscape of interstitial lung disease in idiopathic inflammatory myopathies: state of the art, evidence gaps and a need for clinical trials

Interstitial lung disease (ILD) affects a significant proportion of adults and children with idiopathic inflammatory myopathies (IIM-ILD and JIIM-ILD). Despite its major impact on mortality and therapeutic decision‑making, robust studies and clinical trials to inform evidence‑based practice are strikingly scarce. Heterogeneous clinical manifestations, variable testing practices, and lack of widely accepted nomenclature and standardised endpoint definitions magnify the challenges to design clinical trials for this novel disease subtype.Thus, the Myositis Clinical Trials Consortium (MCTC) developed the IIMILD working group (WG) to bridge the gaps and address the challenges unique to these patients. The IIM-ILD WG will accomplish these objectives by leveraging MCTC's global network of over 960 members, including physicians from multiple specialties, researchers, industry collaborators, and patient-support organisations. Collectively, the paper emphasises the need for structured phenotyping, unified terminology, and validated outcome measures as indispensable prerequisites for designing rigorous, multicentre trials in patients with IIM-ILD and JIIM-ILD. By leveraging the MCTC platform, the IIM-ILD Working Group will accelerate therapeutic development and ultimately improve outcomes for adults and children afflicted with IIM-associated ILD.

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Publication Details

Journal
Clinical and Experimental Rheumatology
Published
2026-02-24
DOI
https://doi.org/10.55563/clinexprheumatol/rsnsu2
Primary Topic
Inflammatory Myopathies and Dermatomyositis
Type
article
Field-Weighted Citation Impact
0.00

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article

Shaping the landscape of interstitial lung disease in idiopathic inflammatory myopathies: state of the art, evidence gaps and a need for clinical trials

Robert W. Hallowell, Narendra Kumar Bagri, Kristin Houghton, Marc H.A. Jansen et al.
Clinical and Experimental Rheumatology
Inflammatory Myopathies and Dermatomyositis
article

Shaping the landscape of interstitial lung disease in idiopathic inflammatory myopathies: state of the art, evidence gaps and a need for clinical trials

Robert W. Hallowell, Narendra Kumar Bagri, Kristin Houghton, Marc H.A. Jansen, Faye Pais, Jisna Paul, Tsung-Yu Lan, C. Papadopoulou, Prateek C. Gandiga, T Kulkarni, Elena K. Joerns, Ho So, Rohit Aggarwal, Brigitte Bader Meunier, Jorge Rojas Serrano, Erin M Wilfong, Vivek Nagaraja, Daphne Rivero Gallegos
article en

Abstract

Interstitial lung disease (ILD) affects a significant proportion of adults and children with idiopathic inflammatory myopathies (IIM-ILD and JIIM-ILD). Despite its major impact on mortality and therapeutic decision‑making, robust studies and clinical trials to inform evidence‑based practice are strikingly scarce. Heterogeneous clinical manifestations, variable testing practices, and lack of widely accepted nomenclature and standardised endpoint definitions magnify the challenges to design clinical trials for this novel disease subtype.Thus, the Myositis Clinical Trials Consortium (MCTC) developed the IIMILD working group (WG) to bridge the gaps and address the challenges unique to these patients. The IIM-ILD WG will accomplish these objectives by leveraging MCTC's global network of over 960 members, including physicians from multiple specialties, researchers, industry collaborators, and patient-support organisations. Collectively, the paper emphasises the need for structured phenotyping, unified terminology, and validated outcome measures as indispensable prerequisites for designing rigorous, multicentre trials in patients with IIM-ILD and JIIM-ILD. By leveraging the MCTC platform, the IIM-ILD Working Group will accelerate therapeutic development and ultimately improve outcomes for adults and children afflicted with IIM-associated ILD.

Clinical and Experimental RheumatologyVol. 44(2)
Emory Healthcare (US), Harvard University (US), University of British Columbia (CA), University of Pittsburgh (US), Chinese University of Hong Kong (HK), Great Ormond Street Hospital for Children NHS Foundation Trust (GB), The Ohio State University Wexner Medical Center (US), University of Alabama at Birmingham (US), University of Florida (US), Massachusetts General Hospital (US), Assistance Publique – Hôpitaux de Paris (FR), Instituto Nacional de Enfermedades Respiratorias (MX), Pulmonary and Allergy Associates (US), Mayo Clinic in Arizona (US), National Taiwan University Hospital (TW), Wilhelmina Children's Hospital (NL), University College London (GB), All India Institute of Medical Sciences (IN), Vanderbilt University Medical Center (US), Florida College (US)
Massachusetts General Hospital, Vanderbilt University, Ohio State University, Emory University, University of Pittsburgh, University of Alabama at Birmingham, University of Alabama, Vanderbilt University Medical Center, University College London, Great Ormond Street Hospital for Children, Chinese University of Hong Kong, National Taiwan University Hospital, National Taiwan University, Assistance publique-Hôpitaux de Paris, Great Ormond Street Institute of Child Health
Good health and well-being
Openalex Percentile: Top 45%
Inflammatory Myopathies and Dermatomyositis
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