Figures
- Figure 1.
Diagnostic algorithm for idiopathic pulmonary fibrosis (IPF). ILD: interstitial lung disease; CTD: connective tissue disease; HRCT: high-resolution computed tomography; UIP: usual interstitial pneumonia. Reproduced from [2] with permission from the publisher.
- Figure 2.
High-resolution computed tomography scan demonstrating a typical example of usual interstitial pneumonia pattern with honeycombing change and traction bronchiectasis, as well as subpleural and basal reticulation. No features are seen suggesting an alternative diagnosis.
- Figure 3.
a, b) Representative examples of the lung biopsies. High-resolution computed tomography scans demonstrating possible usual interstitial pneumonia pattern with subpleural and basal reticulation, and traction bronchiectasis. No features are seen suggesting an alternative diagnosis. Video-assisted surgical lung biopsy demonstrated definite usual interstitial pneumonia pattern.
- Figure 4.
High-resolution computed tomography scans demonstrating patterns not consistent with usual interstitial pneumonia. a) Prominent and diffuse ground-glass attenuation with traction bronchiectasis in a patient with idiopathic nonspecific interstitial pneumonia. b) Ground-glass attenuation with mosaic distribution in a patient with hypersensitivity pneumonitis.
Tables
- Table 1. High-resolution computed tomography criteria for usual interstitial pneumonia (UIP) pattern
UIP pattern (all four features) Possible UIP pattern (all three features) Inconsistent with UIP pattern (any of the features) Subpleural, basal predominance Subpleural, basal predominance Upper or mid-lung predominance Reticular abnormality Reticular abnormality Peribronchovascular predominance Honeycombing with or without traction bronchiectasis Absence of features listed as inconsistent with UIP pattern Extensive ground-glass abnormality (extent > reticular abnormality) Absence of features listed as inconsistent with UIP pattern Profuse micronodules (bilateral, predominantly upper lobes) Discrete cysts (multiple, bilateral, away from areas of honeycombing) Diffuse mosaic attenuation/air-trapping (bilateral and in ≥3 lobes) Consolidation in bronchopulmonary segment(s)/lobe(s) Reproduced from [2] with permission from the publisher.
- Table 2. Diagnosis of idiopathic pulmonary fibrosis (IPF)
HRCT features Pathological features Definite IPF UIP No biopsy Definite UIP Probable UIP Possible UIP Unclassifiable fibrosis Possible UIP Definite UIP Probable UIP Probable IPF Possible UIP Possible UIP Unclassifiable fibrosis Possible IPF Inconsistent with UIP Definite UIP Not IPF UIP Non-UIP Possible UIP Non-UIP Inconsistent with UIP Probable UIP Possible UIP Unclassifiable fibrosis Not UIP The high-resolution computed tomography (HRCT) criteria for usual interstitial pneumonia (UIP) are listed in table 1. A diagnosis of IPF is made when the HRCT feature is associated with one pathological feature.
- Table 3. Chest high-resolution computed tomography methods
Mandatory No injection of contrast medium Inspiratory apnoea slices Axial contiguous or non-reconstructed slices separated by ≤2 cm Slice thickness ≤2 mm Reconstruction field focused on lungs Image acquisition complying with European irradiation norms Archiving of image acquisition of millimetric slices on CD/DVD for further reading Optional Frontal reconstruction in minimal intensity projection, 5–8 mm thick# Images in the prone position, if opacities linked to gravito-dependance hamper the analysis when in the supine position Expiration slices to exclude lobular air trapping¶ Coronal and sagittal reconstructions if volumetric acquisitions are available #: to differentiate bronchiolectasis from honeycombing; ¶: more frequent in patients with hypersensitivity pneumonitis.
- Table 4. Indications and techniques of lung biopsy in patients with suspected idiopathic pulmonary fibrosis (IPF)
Indication Absence of HRCT features sufficient to diagnose IPF# Setting Surgical centre experienced in the management of interstitial lung diseases and in performing video-assisted surgical lung biopsies Technique Video-assisted surgery whenever possible (excluding transbronchial biopsies)¶ Sampling In depth, in several lobes+, avoiding the extremity of the lingula and middle lobe HRCT: high-resolution computed tomography. #: theoretical indication, to be balanced during multidisciplinary team discussion with risk associated with biopsy, age and possible comorbid conditions, and to be discussed with the patient; ¶: to collect samples of an adequate size; +: lesions may differ between the lobes.
- Table 5. Histopathological criteria for usual interstitial pneumonia (UIP) pattern
UIP pattern (all four features) Probable UIP pattern Possible UIP pattern (all three features) Not UIP pattern (any of the features) Evidence of marked fibrosis/architectural distortion±honeycombing in a predominantly subpleural/paraseptal distribution Evidence of marked fibrosis/architectural distortion±honeycombing Patchy or diffuse involvement of lung parenchyma by fibrosis, with or without interstitial inflammation Hyaline membranes# Presence of patchy involvement of lung parenchyma by fibrosis Absence of either patchy involvement or fibroblastic foci, but not both Absence of other criteria for UIP Organising pneumonia#,¶ Presence of fibroblast foci Absence of features against a diagnosis of UIP suggesting an alternate diagnosis Absence of features against a diagnosis of UIP suggesting an alternate diagnosis Granulomas¶ Absence of features against a diagnosis of UIP suggesting an alternate diagnosis OR Honeycomb changes only+ Marked interstitial inflammatory cell infiltrate away from honeycombing Predominant airway-centred changes Other features suggestive of an alternate diagnosis #: can be associated with acute exacerbation of idiopathic pulmonary fibrosis; ¶: an isolated or occasional granuloma and/or a mild component of organising pneumonia pattern may rarely coexist in lung biopsies with an otherwise UIP pattern; +: this scenario usually represents end-stage fibrotic lung disease where honeycombed segments have been sampled but where a UIP pattern might be present in other areas. Such areas are usually represented by overt honeycombing on high-resolution computed tomography (HRCT) and can be avoided by pre-operative targeting of biopsy sites away from these areas using HRCT. Reproduced from [2] with permission from the publisher.
- Table 6. Main examinations that are useful for idiopathic pulmonary fibrosis (IPF) diagnosis and management
At IPF diagnosis Systematic Chest HRCT FVC and DLCO Doppler echocardiography Biology Antinuclear antibodies Anti-citrullinated cyclic peptides antibodies Rheumatoid factor Differential blood cell count C-reactive protein Serum creatinine Transaminases, γ-glutamyltransferase and alkaline phosphatases Occasional Video-assisted surgical lung biopsy (see text) Differential cell count of BAL (see text) TLC and arterial blood gas in room air at rest 6MWT with measurement of percutaneous oxygen saturation Depending on context Genetic testing (see text) Anti-neutrophil cytoplasmic antibodies Anti-SSA and anti-SSB antibodies Anti-centromere and anti-topoisomerase antibodies Anti-Mi-2, anti-U3RNP and anti-ARNt synthetase antibodies Creatine phosphokinase Anti-thyroid antibodies Precipitines (depending on disease presentation) Microbiology of BAL Electrophoresis of serum proteins, immuno-electrophoresis of serum proteins, urine immunofixation and cryoglobulinaemia Exploration for gastro-oesophageal reflux Exploration for sleep apnoea syndrome During follow-up Every 3 to 6 months FVC and DLCO Depending on context Chest HRCT Doppler echocardiography TLC Arterial blood gas in room air Right heart catheterisation (see text) HRCT: high-resolution computed tomography; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide; BAL: bronchoalveolar lavage; TLC: total lung capacity; 6MWT: 6-min walk test.
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Jump To
- Article
- Abstract
- Introduction
- Missions of the committees
- Guidelines development process
- Diagnosis of IPF
- Question 1: What main causes of diffuse interstitial pneumonia should be clinically investigated in patients considered to potentially have IPF?
- Question 2: What biological work-up should be performed in patients considered to have IPF?
- Question 3: Should BAL be performed in patients suspected of having IPF?
- Question 4: When should genetic testing be performed in patients suspected of having IPF?
- Question 5: What pulmonary function tests should be performed at the time of IPF diagnosis?
- Question 6: When is HRCT sufficient for the diagnosis of IPF in patients not subjected to video-assisted SLB?
- Question 7: In what patients should SLB be considered to establish the diagnosis of IPF?
- Question 8: What is the role of multidisciplinary team discussion in the diagnosis of IPF and how is it conducted?
- Question 9: How should prognosis be assessed in IPF patients?
- Treatment of IPF
- Conclusion
- Acknowledgments
- Footnotes
- References
- Figures & Data
- Info & Metrics