Learning objectives
To analyze and exemplify the grey areas between the 4 categories of usual interstitial pneumonia (UIP) CT patterns in the diagnosis criteria for idiopathic pulmonary fibrosis according to the Fleischner Society White Paper.
Define the practical implications of the different categories in follow-up, stressing the importance of multidisciplinary discussion.
Background
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). [1]
In Europe, IPF prevalence ranges from 1.25 to 23.4 cases per 100,000 and the annual incidence between 0.22 and 7.4 per 100,000. IPF prevalence and incidence increase with age, is higher among males and appears to be increasing in recent years. [2]It has a median...
Findings and procedure details
Typical/probable UIP grey area:
One of the grey areas in the diagnosis criteria for idiopathic pulmonary fibrosis according to the Fleischner Society White Paper is the distinction between honeycombing and peripheral traction bronchiolectasis.
T. Watadani et al [10] stated that even experienced radiologists may disagree on the identification of honeycombing, highlighting the difficulties in differentiating the pattern from mimickers such as traction bronchiectasis.
The Fleischner Society White Paper suggests using MinIP reconstructions to help this distinction. Y.Y. Sabri et al. [11] concluded that traction bronchiectasis...
Conclusion
In the appropriate IPF clinical setting, the distinction between “typical” and “probable UIP” CT pattern has limited diagnostic or prognosis implication. IPF diagnosis confidence is higher with “typical UIP” CT pattern.
If the clinical context suggests an alternative diagnosis, biopsy should be considered, despite having either “typical” or “probable UIP” CT pattern.
The other grey area in the Fleischner Society White Paper is the distinction between “indeterminate” and “most consistent with non-IPF”, for which a cut-off point in grading of specific features is yet to...
Personal information and conflict of interest
J. M. Veiga; Lisbon/PT - nothing to disclose C. A. S. Ruano; Lisbon/PT - nothing to disclose O. Fernandes; Lisbon/PT - nothing to disclose A. Borba; Lisbon/PT - nothing to disclose E. Pinto; Lisbon/PT - nothing to disclose L. Figueiredo; Lisbon/PT - nothing to disclose D. Argyropoulou; Almada/PT - nothing to disclose
References
[1] Christenson, M. R. D. (2012). An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Yearbook of Diagnostic Radiology.
[2] Nalysnyk, L., et al (2012). Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature. European Respiratory Review, 21(126), 355–361.
[3] Fabrellas, E. F., et al (2018). Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis. Medical Sciences.
[4] Maher, T. M., et al (2019). Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat. Respiratory Research.
[5] Collins, B. F., et al....
