Inflammatory Myopathies Related Interstitial Lung Disease

Challenges in IIM-ILD and Clinical Trials

  1. The variability in how the disease presents and progresses, with some patients experiencing rapid decline, while others progress more slowly.
  2. The lack of standardized criteria for screening and monitoring ILD further complicates early detection and intervention.
  3. The rarity of IIM-ILD poses difficulties in gathering large patient cohorts for research, limiting the ability to conduct robust clinical trials and identify reliable prognostic markers.

Aims of the IIM-ILD Subsets Working Group:

  1. Improving Early Detection: Develop and promote standardized screening protocols for ILD in patients with IIM, enabling earlier diagnosis and intervention. This will ultimately enable clinical
    trials for patients at earlier stages of ILD, where immunomodulatory therapies might be more effective.
  2. Defining Prognostic Markers: Identify and validate clinical and molecular markers that can predict disease progression and outcomes, guiding enrichment of trial population as well as targeted therapeutic interventions based on molecular signal.
  3. Establishing Collaborative Research Networks: Foster international collaboration to build large, diverse patient registries, allowing for more comprehensive studies which will lead to better understanding of disease subsets, natural history and potential therapeutic targets.
  4. Advancing Therapeutic Strategies: Explore and promote novel therapeutic approaches, including immunosuppressive therapies and targeted treatments aimed at reducing lung inflammation and fibrosis in IIM-ILD.
  5. Developing Reliable and Objective Outcome Measures: Establish outcome measures tailored to monitor ILD progression in IIM patients. These standardized measures will improve both trial design and patient monitoring, ensuring more precise evaluation of treatment responses over time.