Moving beyond usual interstitial pneumonia to define progressive fibrotic interstitial lung disease

Abstract

The spectrum of fibrotic interstitial lung disease (ILD) encompasses dozens of heterogeneous disorders of multiple causes that are characterised by the predominant feature of fibrosis of the lung parenchyma, along with variable degrees of inflammation.1,2 The approach to the classification and understanding of fibrotic ILDs has evolved from the early description of “diffuse interstitial fibrosis of the lungs” by Hamman and Rich3 in the 1930s, to the first international consensus statement by the American Thoracic Society (ATS) and European Respiratory Society (ERS) on the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF), published in 2000 and most recently updated in the form of a clinical practice guideline in 2018.