Table 2. Histopathological Criteria for UIP and NSIP.
UIP* | Probable UIP* | Indeterminate UIP* | Alternative Diagnosis* |
---|---|---|---|
Dense fibrosis with architectural distortion (i.e., destructive scarring and/or honeycombing) | Some histologic features from UIP (column 1) are present but to an extent that preludes a definite diagnosis of UIP/IPF | Fibrosis with or without architectural distortion with features favoring either a pattern other than UIP or features favoring UIP secondary to another cause | Features of other histologic patterns of IIPs (e.g., absence of fibroblast foci or loose fibrosis) in all biopsies |
Predominantly subpleural and/or paraseptal distribution of fibrosis | AND | ||
Absence of features to suggest an alternate diagnosis | Histologic findings indicative of other diseases (e.g., hypersensitivity pneumonitis, Langerhans cell histiocytosis, sarcoidosis, LAM) | ||
Some histologic features from UIP (column 1), but with other features suggesting an alternative diagnosis | |||
Patchy involvement of lung parenchyma by fibrosis | OR | ||
Honeycombing only | |||
Fibroblast foci | |||
Absence of features to suggest an alternate diagnosis | |||
Fibrosing NSIP† | Cellular NSIP† | ||
Key Features | Pertinent Negative Factors | Key Features | Pertinent Negative Factors |
Dense or loose interstitial fibrosis with uniform appearance | Temporal heterogeneity | Mild to moderate interstitial chronic inflammation | Dense interstitial fibrosis |
Fibroblastic foci with dense fibrosis | Prominence of organizing pneumonia | ||
Type II pneumocyte hyperplasia in areas of inflammation | |||
Frequent preservation of lung architecture | |||
Findings above are especially important in case with patchy involvement and subpleural or paraseptal distribution | Diffuse severe alveolar septal inflammation | ||
Mild or moderate interstitial chronic inflammation |
*Raghu et al. Am J Respir Crit Care Med 2018;198:e44-e68 [8], †Travis et al. Am J Respir Crit Care Med 2008;177:1338-1347 [37]. IIP = idiopathic interstitial pneumonia, IPF = idiopathic interstitial fibrosis, LAM = lymphangioleiomyomatosis, NSIP = nonspecific interstitial pneumonia, UIP = usual interstitial pneumonia