Diagnosis of IPF based on the combination of high resolution computed tomography and surgical lung biopsy (requires multidisciplinary discussion)

Histopathology patterns and features
UIP	Probable UIP	Indeterminate for UIP	Alternative diagnosis
Dense fibrosis with architectural distortion (ie, destructive scarring and/or honeycombing) Predominant subpleural and/or paraseptal distribution of fibrosis Patchy involvement of lung parenchyma by fibrosis Fibroblast foci Absence of features to suggest an alternate diagnosis	Some histologic features from column 1 are present but to an extent that precludes a definite diagnosis of UIP/IPF *AND* Absence of features to suggest an alternative diagnosis OR Honeycombing only	Fibrosis with or without architectural distortion, with features favoring either a pattern other than UIP or features favoring UIP secondary to another cause* Some histologic features from column 1, but with other features suggesting an alternative diagnosis^¶	Features of other histologic patterns of IIPs (eg, absence of fibroblast foci or loose fibrosis) in all biopsies Histologic findings indicative of other diseases (eg, hypersensitivity pneumonitis, Langerhans cell histiocytosis, sarcoidosis, LAM)
Idiopathic pulmonary fibrosis (IPF) diagnosis on the basis of high-resolution computed tomography (HRCT) and biopsy patterns, developed using consensus by discussion
IPF suspected^Δ		Histopathology pattern^◊
IPF suspected^Δ		UIP	Probable UIP	Indeterminate for UIP or biopsy not performed	Alternative diagnosis
HRCT pattern	UIP	IPF	IPF	IPF	Non-IPF diagnosis
	Probable UIP	IPF	IPF	IPF (likely)^§	Non-IPF diagnosis
	Indeterminate	IPF	IPF (likely)^§	Indeterminate^¥	Non-IPF diagnosis
	Alternative diagnosis	IPF (likely)^§	Indeterminate^¥	Non-IPF diagnosis	Non-IPF diagnosis

IPF: idiopathic pulmonary fibrosis; UIP: usual interstitial pneumonia; IIP: idiopathic interstitial pneumonia; LAM: lymphangioleiomyomatosis; HRCT: high resolution computed tomography.

* Granulomas, hyaline membranes (other than when associated with acute exacerbation of IPF, which may be the presenting manifestation in some patients), prominent airway-centered changes, areas of interstitial inflammation lacking associated fibrosis, marked chronic fibrous pleuritis, organizing pneumonia. Such features may not be overt or easily seen to the untrained eye and often need to be specifically sought.

¶ Features that should raise concerns about the likelihood of an alternative diagnosis include a cellular inflammatory infiltrate away from areas of honeycombing, prominent lymphoid hyperplasia including secondary germinal centers, and a distinctly bronchiolocentric distribution that could include extensive peribronchiolar metaplasia.

Δ "Clinically suspected of having IPF" is defined as unexplained patterns of bilateral pulmonary fibrosis on chest radiography or chest computed tomography, bibasilar inspiratory crackles, and age >60 years. Middle-aged adults (>40 and <60 years old) can rarely present with otherwise similar clinical features, especially in patients with features suggesting familial pulmonary fibrosis.

◊ Diagnostic confidence may need to be downgraded if histopathological assessment is based on transbronchial lung cryobiopsy given the smaller biopsy size and greater potential for sampling error compared with surgical lung biopsy.

§ IPF is the likely diagnosis when any of the following features are present:

Moderate to severe traction bronchiectasis and/or bronchiolectasis (defined as mild traction bronchiectasis and/or bronchiolectasis in four or more lobes, including the lingula as a lobe, or moderate to severe traction bronchiectasis in two or more lobes) in a man >50 years old or in a woman >60 years old
Extensive (>30%) reticulation on HRCT and age >70 years
Increased neutrophils and/or absence of lymphocytosis in BAL fluid
Multidisciplinary discussion produces a confident diagnosis of IPF

¥ Indeterminate for IPF:

Without an adequate biopsy remains indeterminate
With an adequate biopsy may be reclassified to a more specific diagnosis after multidisciplinary discussion and/or additional consultation

IPF diagnosis based upon HRCT and biopsy patterns reprinted with permission of the American Thoracic Society. Copyright © 2022 American Thoracic Society. All rights reserved. From: Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2022; 205(9):e18-e47. The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

Graphic 107408 Version 4.0

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Diagnosis of IPF based on the combination of high resolution computed tomography and surgical lung biopsy (requires multidisciplinary discussion)