Updates in the diagnosis of interstitial lung disease: evolving concepts and terminology

Interstitial lung diseases (ILDs) comprise a heterogeneous group of disorders that involve the interstitium and may be of identified cause or idiopathic.

This review highlights updates in ILD classification and a variety of diagnostic tools. Syndromic classifications including progressive pulmonary fibrosis, systemic autoimmune rheumatic disease-associated ILD, interstitial pneumonia with autoimmune features, and interstitial lung abnormalities are discussed with attention to their clinical utility and limitations. Updates in molecular testing, including genetic testing for telomere-related disorders and telomere length, are discussed along with a brief review of radiomics and its utility in ILD classification and prognostication. We conclude by examining the central role of multidisciplinary discussion in integrating these data into a robust clinical diagnosis.

Diagnostic and classification schemes will continue to evolve with diagnostic standards shifting away from lung biopsy to multidisciplinary discussion. Further advances in 'omics' including radiomics and molecular testing will likely aid in classification of disease.