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Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines

Vincent Cottin, Bruno Crestani, Dominique Valeyre, Benoit Wallaert, Jacques Cadranel, Jean-Charles Dalphin, Philippe Delaval, Dominique Israel-Biet, Romain Kessler, Martine Reynaud-Gaubert, Bernard Aguilaniu, Benoit Bouquillon, Philippe Carré, Claire Danel, Jean-Baptiste Faivre, Gilbert Ferretti, Nicolas Just, Serge Kouzan, François Lebargy, Sylvain Marchand-Adam, Bruno Philippe, Grégoire Prévot, Bruno Stach, Françoise Thivolet-Béjui, Jean-François Cordier, the French National Reference Centre and the Network of Competence Centres for Rare Lung Diseases
European Respiratory Review 2014 23: 193-214; DOI: 10.1183/09059180.00001814
Vincent Cottin
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Bruno Crestani
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Dominique Valeyre
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Benoit Wallaert
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Jacques Cadranel
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Jean-Charles Dalphin
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Philippe Delaval
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Dominique Israel-Biet
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Romain Kessler
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Martine Reynaud-Gaubert
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Bernard Aguilaniu
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Benoit Bouquillon
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Philippe Carré
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Claire Danel
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Jean-Baptiste Faivre
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Gilbert Ferretti
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Nicolas Just
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Serge Kouzan
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François Lebargy
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Sylvain Marchand-Adam
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Bruno Philippe
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Grégoire Prévot
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Bruno Stach
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Françoise Thivolet-Béjui
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Jean-François Cordier
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  • Figure 1.
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    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.
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    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.
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    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.
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    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

  • Figures
  • Additional Files
  • 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 predominanceSubpleural, basal predominanceUpper or mid-lung predominance
    Reticular abnormalityReticular abnormalityPeribronchovascular predominance
    Honeycombing with or without traction bronchiectasisAbsence of features listed as inconsistent with UIP patternExtensive ground-glass abnormality (extent > reticular abnormality)
    Absence of features listed as inconsistent with UIP patternProfuse 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 featuresPathological features
    Definite IPFUIPNo biopsy
    Definite UIP
    Probable UIP
    Possible UIP
    Unclassifiable fibrosis
    Possible UIPDefinite UIP
    Probable UIP
    Probable IPFPossible UIPPossible UIP
    Unclassifiable fibrosis
    Possible IPFInconsistent with UIPDefinite UIP
    Not IPFUIPNon-UIP
    Possible UIPNon-UIP
    Inconsistent with UIPProbable 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)
    IndicationAbsence of HRCT features sufficient to diagnose IPF#
    SettingSurgical centre experienced in the management of interstitial lung diseases and in performing video-assisted surgical lung biopsies
    TechniqueVideo-assisted surgery whenever possible (excluding transbronchial biopsies)¶
    SamplingIn 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 patternPossible UIP pattern (all three features)Not UIP pattern (any of the features)
    Evidence of marked fibrosis/architectural distortion±honeycombing in a predominantly subpleural/paraseptal distributionEvidence of marked fibrosis/architectural distortion±honeycombingPatchy or diffuse involvement of lung parenchyma by fibrosis, with or without interstitial inflammationHyaline membranes#
    Presence of patchy involvement of lung parenchyma by fibrosisAbsence of either patchy involvement or fibroblastic foci, but not bothAbsence of other criteria for UIPOrganising pneumonia#,¶
    Presence of fibroblast fociAbsence of features against a diagnosis of UIP suggesting an alternate diagnosisAbsence of features against a diagnosis of UIP suggesting an alternate diagnosisGranulomas¶
    Absence of features against a diagnosis of UIP suggesting an alternate diagnosisOR 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.

Additional Files

  • Figures
  • Tables
  • Disclosures

    Files in this Data Supplement:

    • B. Aguilaniu
    • G. Prevot
    • D. Valeyre
    • B. Wallaert
    • J. Cadranel
    • J-F. Cordier
    • V. Cottin
    • B. Crestani
    • J-C. Dalphin
    • P. Delaval
    • G. Ferretti
    • R. Kessler
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Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines
Vincent Cottin, Bruno Crestani, Dominique Valeyre, Benoit Wallaert, Jacques Cadranel, Jean-Charles Dalphin, Philippe Delaval, Dominique Israel-Biet, Romain Kessler, Martine Reynaud-Gaubert, Bernard Aguilaniu, Benoit Bouquillon, Philippe Carré, Claire Danel, Jean-Baptiste Faivre, Gilbert Ferretti, Nicolas Just, Serge Kouzan, François Lebargy, Sylvain Marchand-Adam, Bruno Philippe, Grégoire Prévot, Bruno Stach, Françoise Thivolet-Béjui, Jean-François Cordier, the French National Reference Centre and the Network of Competence Centres for Rare Lung Diseases
European Respiratory Review Jun 2014, 23 (132) 193-214; DOI: 10.1183/09059180.00001814

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Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines
Vincent Cottin, Bruno Crestani, Dominique Valeyre, Benoit Wallaert, Jacques Cadranel, Jean-Charles Dalphin, Philippe Delaval, Dominique Israel-Biet, Romain Kessler, Martine Reynaud-Gaubert, Bernard Aguilaniu, Benoit Bouquillon, Philippe Carré, Claire Danel, Jean-Baptiste Faivre, Gilbert Ferretti, Nicolas Just, Serge Kouzan, François Lebargy, Sylvain Marchand-Adam, Bruno Philippe, Grégoire Prévot, Bruno Stach, Françoise Thivolet-Béjui, Jean-François Cordier, the French National Reference Centre and the Network of Competence Centres for Rare Lung Diseases
European Respiratory Review Jun 2014, 23 (132) 193-214; DOI: 10.1183/09059180.00001814
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  • 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
  • PDF

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  • Interstitial and orphan lung disease
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