11
Figure 8. IPF Diagnosis Based Upon HRCT and Biopsy
Patterns
IPF suspected* Histopathology pattern
UIP
Probable
UIP
Indeterminate
for UIP
Alternative
diagnosis
HRCT
pattern
UIP IPF IPF IPF Non-IPF dx
Probable
UIP
IPF IPF IPF (Likely)** Non-IPF dx
Indeterminate
for UIP
IPF IPF
(Likely)**
Indeterminate
for IPF***
Non-IPF dx
Alternative
diagnosis
IPF
(Likely)**/
non-IPF dx
Non-IPF dx Non-IPF dx Non-IPF dx
* "Clinically suspected of having IPF" = unexplained symptomatic or asymptomatic patterns of
bilateral pulmonary fibrosis on a chest radiograph or chest computed tomography, bibasilar
inspiratory crackles, and age greater than 60 years. (Middle-aged adults [>40 yr and <60 yr],
especially patients with risks for familial pulmonary fibrosis, can rarely present with the otherwise
same clinical scenario as the typical patient older than 60 years.)
**IPF is the likely diagnosis when any of the following features are present:
• Moderate-to-severe traction bronchiectasis/bronchiolectasis (defined as mild traction
bronchiectasis/bronchiolectasis in four or more lobes including the lingual as a lobe, or moderate
to severe traction bronchiectasis in two or more lobes) in a man over age 50 years or in a woman
over age 60 years
• Extensive (>30%) reticulation on HRCT and an age >70 years
• Increased neutrophils and/or absence of lymphocytosis in BAL fluid
• Multidisciplinary discussion reaches a confident diagnosis of IPF.
***Indeterminate for IPF
• Without an adequate biopsy is unlikely to be IPF
• With an adequate biopsy may be reclassified to a more specific diagnosis aer multidisciplinary
discussion and/or additional consultation.
