Interstitial lung disease encompasses hundreds of diseases. the one that is being talked about more is idiopathic interstitial pneumonia, with a focus on idiopathic pulmonary fibrosis because of its higher incidence (compared to other similar diseases). So look at the chunk of articles on interstitial lung disease in recent years that deal with them. the consensus on IIP came out in 2000. it was reworked 2 years later, with a separate modification of IPF treatment in 2011, and by next year (2013), another adjustment to the classification of IIP into chronic fibrogenic IID, rare IIP, unclassified IIP, etc. Throughout the years, several changes to IIP have been made, mostly limited to the conceptual level. Jiang Handong, Department of Respiratory Medicine, Shanghai Renji Hospital
If we look at the recent studies, we can easily find that the concept of “idiopathic” is actually derived from “uncharacterized”, i.e., not manifested at the time of disease diagnosis or not yet available for examination. Many IPFs were later found to resemble occupational lung diseases, and about 40-50% of iNSIPs were later derived from systemic immune diseases. How many of these “idiopathic” cases were later removed from the label?
If we look at IPF, we know that there is often uncertainty in the imaging, so if we rely on the new 2011 ATS/ERS consensus on IPF, we may miss some ILDs that might otherwise be effective with hormones and be misdiagnosed as IPF due to the uncertainty in the imaging. There are fewer of them.
Therefore, clinical diagnosis of IPF cases should closely mention which ILDs that may be hormone-sensitive, such as iNSIP.