Demystifying idiopathic interstitial pneumonia: time for more etiology-focused nomenclature in interstitial lung disease. Issue 2 (1st February 2022)

• Record Type: Journal Article
• Authors: Todd, Nevins W.; Atamas, Sergei P.; Hines, Stella E.; Luzina, Irina G.; Shah, Nirav G.; Britt, Edward J.; Ghio, Andrew J.; Galvin, Jeffrey R.
• Is Part Of: Expert review of respiratory medicine. Volume 16:Issue 2(2022)
• Page Start: 235
• Page End: 245
• Publication Date: 2022-02-01
• Subjects: Idiopathic interstitial pneumonia -- interstitial lung disease -- idiopathic pulmonary fibrosis -- usual interstitial pneumonia -- nonspecific interstitial pneumonia -- organizing pneumonia; Respiratory organs -- Diseases -- Periodicals; Respiratory organs -- Diseases -- Treatment -- Periodicals; 616.2005
• DOI: 10.1080/17476348.2022.2030710
• Languages: English
• ISSNs: 1747-6348

Abstract

ABSTRACT: Introduction: A major focus of interstitial lung disease (ILD) has centered on disorders termed idiopathic interstitial pneumonias (IIPs) which include, among others, idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, and respiratory bronchiolitis-interstitial lung disease. Areas Covered: We review the radiologic and histologic patterns for the nine disorders classified by multidisciplinary approach as IIP, and describe the remarkable amount of published epidemiologic, translational, and molecular studies demonstrating their associations with numerous yet definitive environmental exposures, occupational exposures, pulmonary diseases, systemic diseases, medication toxicities, and genetic variants. Expert Opinion: In the 21st century, these disorders termed IIPs are rarely idiopathic, but rather are well-described radiologic and histologic patterns of lung injury that are associated with a wide array of diverse etiologies. Accordingly, the idiopathic nomenclature is misleading and confusing, and may also promote a lack of inquisitiveness, suggesting the end rather than the beginning of a thorough diagnostic process to identify ILD etiology and initiate patient-centered management. A shift toward more etiology-focused nomenclature will be beneficial to all, including patients hoping for better life quality and disease outcome, general medicine and pulmonary physicians furthering their ILD knowledge, and expert ILD clinicians and researchers who are advancing the ILD field.