Learning objectives
1.
To know the updated diagnostic guidelines of idiopathic pulmonary fibrosis (IPF) published in 2018: Fleischner Society White Paper Consensus Statement and ATS/ERS/JRS/ALAT guideline.
2.
To describe the updated diagnostic categories of UIP based on HRCT patterns.
3.
To explain the role of the working multidisciplinary diagnosis of IPF in different clinical scenarios.
Background
The interstitial lung diseases (ILDs) are a complex group of conditions that present with nonspecific symptoms.
One of the more common and the most fatal of the ILDs is idiopathic pulmonary fibrosis (IPF).
IPF is a specific form of chronic,
progressive,
fibrosing interstitial pneumonia of unknown cause that occurs primarily in older adults.
It is defined by the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP).
To achieve an accurate diagnosis of ILD,
the multidisciplinary review is often necessary.
The first evidence-based guidelines for...
Imaging findings OR procedure details
IPF is a chronic fibrotic lung disease limited to the lungs characterized by patchy,
peripheral lobular fibrosis that progresses to “honeycombing” and end-stage fibrosis over the course of years.
Updates to diagnostic guidelines for IPF
The updated ATS/ERS/JRS/ALAT guideline and Fleischner Society statement reach similar conclusions and recommendations with only small differences.
We review the main points of the updated diagnostic guidelines for IPF.
1- Clinical aspects
Both documents emphasize the pivotal role of the clinician in evaluating patients suspected to have IPF,
particularly excluding...
Conclusion
New diagnostic criteria and approach to the diagnosis of IPF have been recently introduced.
Possible UIP CT pattern has been removed and two new patterns have been described: Probable UIP and indeterminate for UIP patterns.
IPF diagnosis can be confidently made with a clinical context of IPF and a CT pattern of typical or probable UIP,
according to Fleischner recommendation.
In the ATS/ERS/JRS/ALAT guideline,
surgical lung biopsy is still considered in probable UIP CT pattern as a conditional recommendation.
The role of the radiologist in...
References
1- Raghu G 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 Sep 1;198(5):e44-e68.
2- Lynch DA,
Sverzellati N,
Travis WD,
Brown KK,
Colby TV,
Galvin JR,
Goldin JG,
Hansell DM,
Inoue Y,
Johkoh T,
Nicholson AG,
Knight SL,
Raoof S,
Richeldi L,
Ryerson CJ,
Ryu JH,
Wells AU.
Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper....