Reviews
Shaping the landscape of interstitial lung disease in idiopathic inflammatory myopathies: state of the art, evidence gaps and a need for clinical trials
D. Rivero Gallegos1, F.M. Pais2, T. Lan3, C. Papadopoulou4, E.K. Joerns5, J. Paul6, J. Rojas Serrano7, T. Kulkarni8, P.C. Gandiga9, H. So10, K. Houghton11, N. Bagri12, M. Jansen13, B. Bader Meunier14, E.M. Wilfong15, R. Aggarwal16, R.W. Hallowell17, V. Nagaraja18
- Instituto Nacional de Enfermedades Respiratorias (INER), Mexico City, Mexico.
- Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, College of Medicine, Gainesville, FL, USA.
- National Taiwan University Hospital, Taipei, Taiwan.
- Rheumatology Department, Great Ormond Street Hospital for Children NHS Foundation Trust and Great Ormond Street Institute of Child Health, University College London, UK.
- Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
- Division of Rheumatology and Immunology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
- Instituto Nacional de Enfermedades Respiratorias (INER), Mexico City, Mexico.
- Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, The University of Alabama at Birmingham, AL, USA.
- Division of Rheumatology, Emory Healthcare, Atlanta, GA, USA.
- Division of Medicine and Therapeutics, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong SAR, China.
- Division of Rheumatology, Department of Paediatrics, University of British Columbia, Vancouver, Canada.
- Division of Paediatric Rheumatology, Department of Paediatrics, All India Institute of Medical Sciences, New Delhi, India.
- Department of Paediatrics Immunology and Rheumatology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, The Netherlands.
- Meunier Department of Paediatric Immunology and Rheumatology, Necker Hospital, Assistance Publique–Hôpitaux de Paris, France.
- Division of Rheumatology and Immunology, Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
- Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, PA, USA.
- Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
- Division of Rheumatology, Mayo Clinic Arizona, Scottsdale, AZ, USA. nagaraja.vivek@mayo.edu
CER19374
2026 Vol.44, N°2
PI 0368, PF 0375
Reviews
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PMID: 41738254 [PubMed]
Received: 01/10/2025
Accepted : 23/12/2025
In Press: 24/02/2026
Published: 24/02/2026
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.
