Present and future perspectives in early diagnosis and monitoring for progressive fibrosing interstitial lung diseases.

Progressive fibrosing interstitial lung diseases (PF-ILDs) represent a group of conditions of both known and unknown origin which continue to worsen despite standard treatments, leading to respiratory failure and early mortality. Given the potential to slow down progression by initiating antifibrotic therapies where appropriate, there is ample opportunity to implement innovative strategies for early diagnosis and monitoring with the goal of improving clinical outcomes. Early diagnosis can be facilitated by standardizing ILD multidisciplinary team (MDT) discussions, implementing machine learning algorithms for chest computed-tomography quantitative analysis and novel magnetic-resonance imaging techniques, as well as measuring blood biomarker signatures and genetic testing for telomere length and identification of deleterious mutations in telomere-related genes and other single-nucleotide polymorphisms (SNPs) linked to pulmonary fibrosis such as rs35705950 in the MUC5B promoter region. Assessing disease progression in the post COVID-19 era also led to a number of advances in home monitoring using digitally-enabled home spirometers, pulse oximeters and other wearable devices. While validation for many of these innovations is still in progress, significant changes to current clinical practice for PF-ILDs can be expected in the near future.

Diagnosing interstitial lung disease by multidisciplinary discussion: A review.

The multidisciplinary meeting (MDM) has been endorsed in current international consensus guidelines as the gold standard method for diagnosis of interstitial lung disease (ILD). In the absence of an accurate and reliable diagnostic test, the agreement between multidisciplinary meetings has been used as a surrogate marker for diagnostic accuracy. Although the ILD MDM has been shown to improve inter-clinician agreement on ILD diagnosis, result in a change in diagnosis in a significant proportion of patients and reduce unclassifiable diagnoses, the ideal form for an ILD MDM remains unclear, with constitution and processes of ILD MDMs varying greatly around the world. It is likely that this variation of practice contributes to the lack of agreement seen between MDMs, as well as suboptimal diagnostic accuracy. A recent Delphi study has confirmed the essential components required for the operation of an ILD MDM. The ILD MDM is a changing entity, as it incorporates new diagnostic tests and genetic markers, while also adapting in its form in response to the obstacles of the COVID-19 pandemic. The aim of this review was to evaluate the current evidence regarding ILD MDM and their role in the diagnosis of ILD, the practice of ILD MDM around the world, approaches to ILD MDM standardization and future directions to improve diagnostic accuracy in ILD.