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Lancet Respir Med. 2018 Feb;6(2):138-153. doi: 10.1016/S2213-2600(17)30433-2. Epub 2017 Nov 15.

Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper.

Author information

1
Department of Radiology, National Jewish Health, Denver, CO, USA. Electronic address: lynchd@njhealth.org.
2
Department of Medicine and Surgery, University of Parma, Parma, Italy.
3
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
4
Department of Medicine, National Jewish Health, Denver, CO, USA.
5
Department of Pathology, Mayo Clinic Scottsdale, Scottsdale, AZ, USA.
6
Department of Radiology, University of Maryland, Baltimore, MD, USA.
7
Department of Radiology, David Geffen School of Medicine at UCLA, Santa Monica, CA, USA.
8
Department of Radiology, Royal Brompton and Hospital NHS Foundation Trust and National Heart and Lung Institute, Imperial College, London, UK.
9
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Osaka, Japan.
10
Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Itami, Japan.
11
Department of Histopathology, Royal Brompton and Hospital NHS Foundation Trust and National Heart and Lung Institute, Imperial College, London, UK.
12
Library and Knowledge Services, National Jewish Health, Denver, CO, USA.
13
Division of Pulmonary Medicine, Lenox Hill Hospital, New York, NY, USA.
14
Unità Operativa Complessa di Pneumologia, Agostino Gemelli University Hospital of the Catholic University of the Sacred Heart, Rome, Italy.
15
Department of Medicine, University of British Columbia and St Paul's Hospital, Vancouver, BC, Canada.
16
Division of Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, Rochester, MN, USA.
17
Department of Respiratory Medicine, Royal Brompton and Hospital NHS Foundation Trust and National Heart and Lung Institute, Imperial College, London, UK.

Abstract

This Review provides an updated approach to the diagnosis of idiopathic pulmonary fibrosis (IPF), based on a systematic search of the medical literature and the expert opinion of members of the Fleischner Society. A checklist is provided for the clinical evaluation of patients with suspected usual interstitial pneumonia (UIP). The role of CT is expanded to permit diagnosis of IPF without surgical lung biopsy in select cases when CT shows a probable UIP pattern. Additional investigations, including surgical lung biopsy, should be considered in patients with either clinical or CT findings that are indeterminate for IPF. A multidisciplinary approach is particularly important when deciding to perform additional diagnostic assessments, integrating biopsy results with clinical and CT features, and establishing a working diagnosis of IPF if lung tissue is not available. A working diagnosis of IPF should be reviewed at regular intervals since the diagnosis might change. Criteria are presented to establish confident and working diagnoses of IPF.

PMID:
29154106
DOI:
10.1016/S2213-2600(17)30433-2
[Indexed for MEDLINE]
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