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, ro-bust studies and clinical trials to inform evidence-based practice are strikingly scarce. Heterogeneous clinical manifes-tations, variable testing practices, and lack of widely accepted nomenclature and standardised endpoint definitions magnify the challenges to design clini-cal trials for this novel disease subtype. Thus, the Myositis Clinical Trials Con-sortium (MCTC) developed the IIM-ILD working group (WG) to bridge the gaps and address the challenges unique to these patients. The IIM-ILD WG will accomplish these objectives by lever-aging 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 struc-tured phenotyping, unified terminology, and validated outcome measures as in-dispensable prerequisites for designing rigorous, multicentre trials in patients with IIM-ILD and JIIM-ILD. By lever-aging the MCTC platform, the IIM-ILD Working Group will accelerate thera-peutic development and ultimately im-prove outcomes for adults and children afflicted with IIM-associated ILD.