CHEST Curriculum Pathway for Interstitial Lung Disease: Sample Content

IPF is a disease of unknown cause, occurring primarily in older adults, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). UIP can be diagnosed without a lung biopsy when typical features are seen on HRCT scan of the chest. These CT scan features include bilateral reticular opacities (choice B is incorrect), honeycombing (choice C is incorrect), subpleural/basal predominance of abnormalities, and the absence of findings that are inconsistent with the UIP pattern.

The CT scan features that are inconsistent with the UIP pattern include diffuse mosaic attenuation, upper or midlung predominance, peribronchovascular predominance, predominant ground-glass opacities (choice A is correct), profuse micronodules, discrete cysts, and consolidative opacities. The histopathologic features of UIP include dense fibrosis/architectural distortion ± honeycombing, predominantly subpleural and/or paraseptal distribution, patchy fibrosis, fibroblast foci, and absence of features suggesting alternative diagnoses (eg, granulomas).

Raghu G, Remy-Jardin M, Myers JL, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. PubMed

Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6(2):138-153. PubMed