Connective Tissue Disease-Associated Interstitial Lung Disease: Unmet Needs and Challenges

chest x-ray
chest x-ray
The American College of Rheumatology and Association of Physicians of Great Britain and Ireland have highlighted unmet needs and challenges of connective tissue disease-associated interstitial lung disease.

The American College of Rheumatology and Association of Physicians of Great Britain and Ireland have identified a variety of potential research initiatives to address the unmet needs and challenges of connective tissue disease-associated interstitial lung disease (CTD-ILD).

A multidisciplinary panel of international clinicians specializing in pulmonology, rheumatology, thoracic radiology, and lung pathology convened for a 1-day summit on CTD-ILD to highlight key clinical and research aspects of CTD-ILD, identify unmet needs, and outline future research goals regarding the intersection of these diseases. A summary of the proceedings has been published in Arthritis & Rheumatology.

“Optimal care of patients with CTD-associated ILD requires collaboration and close interaction by the rheumatology and pulmonology communities,” the authors wrote. “Rheumatologists have begun to improve their surveillance for lung involvement in patients with CTD, though clear guidelines (and training) have been lacking. Pulmonologists evaluating patients with ILD have become more attuned to the demographic, historical, and phenotypic features that may suggest an underlying CTD, though their level of expertise with that evaluation varies widely.”

A summary of proposed future directions in CTD-ILD is as follows:

  • International guidelines are needed to standardize clinical, radiologic, histopathologic, and biologic parameters for the diagnosis and classification of CTD-ILD.
  • Multicenter global clinical networks of well-defined disease groups are warranted to define the natural history of CTD-ILD, including collections of phenotypic, physiologic, radiologic, genomic, and biologic data.
  • Multidisciplinary clinics for rheumatology, pulmonology, and additional healthcare providers are needed to enhance CTD-ILD care.
  • Cross-disciplinary fellowship clinical training opportunities should be developed for medical graduates.
  • Precision medicine-based biomarker platforms are needed to guide optimal therapies in individual patients.
  • Early detection strategies need to be developed and utilized to identify ILD earlier and predict patients who are at highest risk for disease progression.
  • ILD imaging repositories need to be generated across the spectrum of CTD-ILD that correlate with histopathologic specimens, and cryobiopsy techniques should be refined to enhance the availability of parenchymal lung tissue specimens.

Related Articles

The panel also identified unmet needs of clinical and translational research in CTD-ILD. Clinical trials of future interventions in CTD-ILD should include:

  • Validated CTD-specific trial end points
  • Novel technologies to validate quality of life end points and patient-reported outcome measures
  • Composite end points specific to CTD-ILD
  • An integrated clinical, radiologic, laboratory, and biologic database solution that aligns large data sets and allows maximum interrogation

Furthermore, to optimize translational research in CTD-ILD, shared national and international registries with biologic repositories should be developed and new quality of life measures should be created. The panel also noted that funding and research efforts to develop better animal models of CTD-ILD are needed.

follow @RheumAdvisor


Fischer A, Strek ME, Cottin V, et al. Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease-Associated Interstitial Lung Disease Summit: a multidisciplinary approach to address challenges and opportunities [published online January 3, 2019]. Arthritis Rheumatol. doi:10.1002/art40769