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.
Chronic respiratory disease
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Authors

Rivero Gallegos Rivero Gallegos, Pais Pais, Lan Lan, Papadopoulou Papadopoulou, Joerns Joerns, Paul Paul, Rojas Serrano Rojas Serrano, Kulkarni Kulkarni, Gandiga Gandiga, So So, Houghton Houghton, Bagri Bagri, Jansen Jansen, Bader Meunier Bader Meunier, Wilfong Wilfong, Aggarwal Aggarwal, Hallowell Hallowell, Nagaraja Nagaraja
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