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Managing diagnostic procedures in idiopathic pulmonary fibrosis

Athol U. Wells
European Respiratory Review 2013 22: 158-162; DOI: 10.1183/09059180.00001213
Athol U. Wells
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK
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  • For correspondence: A.Wells@rbht.nhs.uk
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    Figure 1.

    Classification of interstitial lung disease. EG: eosinophilic granulomatosis; EP: eosinophilic pneumonia; LAM: lymphangioleiomyomatosis. Reproduced and modified from [1] with permission from the publisher.

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    Figure 2.

    2011 American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association diagnostic algorithm for idiopathic pulmonary fibrosis (IPF). ILD: interstitial lung disease; HRCT: high-resolution computed tomography; UIP: usual interstitial pneumonia; MDD: multidisciplinary discussion. Reproduced and modified from [11] with permission from the publisher.

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  • Table 1. Clinical conditions associated with a histopathological pattern of usual interstitial pneumonia
    Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis
    Collagen vascular disease
    Drug toxicity
    Chronic hypersensitivity pneumonitis
    Asbestosis
    Familial idiopathic pulmonary fibrosis
    Hermansky–Pudlak syndrome
    • Reproduced from [1] with permission from the publisher.

  • Table 2. 2002 American Thoracic Society/European Respiratory Society criteria for the diagnosis of idiopathic pulmonary fibrosis
    Major criteria
        Exclusion of other known causes of ILD, such as certain drug toxicities, environmental exposures and connective tissue diseases
        Abnormal pulmonary function studies that include evidence of restriction (reduced VC, often with an increased FEV1/FVC ratio) and impaired gas exchange (increased PA–aO2, decreased PaO2 with rest or exercise, or decreased DLCO)
        Bibasilar reticular abnormalities with minimal ground-glass opacities on HRCT
        Transbronchial lung biopsy or BAL showing no features to support an alternative diagnosis
    Minor criteria
        Age >50 years
        Insidious onset of otherwise unexplained dyspnoea on exertion
        Duration of illness >3 months
        Bibasilar, inspiratory crackles (dry or “Velcro”-type in quality)
    • In the immunocompetent adult, the presence of all of the major diagnostic criteria as well as at least three of the four minor criteria increases the likelihood of a correct clinical diagnosis of idiopathic pulmonary fibrosis. See figure 2 for the updated 2011 criteria. ILD: interstitial lung disease; VC: vital capacity; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; PA–aO2: alveolar–arterial oxygen tension difference; PaO2: arterial oxygen tension; DLCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; BAL: bronchoalveolar lavage. Data from [10].

  • Table 3. Radiological features of idiopathic pulmonary fibrosis
    UIP pattern (all four features)Possible UIP pattern (all three features)Inconsistent with UIP pattern (any one of seven features)
    Subpleural basal predominanceSubpleural basal predominanceUpper or mid-lung predominance
    Peribronchovascular predominance
    Reticular abnormalityReticular abnormalityExtensive ground-glass abnormality (extent greater than reticular abnormality)
    Profuse micronodules (bilateral, predominantly upper lobes)
    Honeycombing with or without traction bronchiectasisDiscrete cysts (multiple bilateral, away from areas of honeycombing)
    Diffuse mosaic attenuation/air trapping (bilateral in three or more lobes)
    Absence of features listed as inconsistent with UIP patternAbsence of features listed as inconsistent with UIP patternConsolidation in bronchopulmonary segment(s)/lobe(s)
    • UIP: usual interstitial pneumonia. Reproduced and modified from [11] with permission from the publisher.

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Managing diagnostic procedures in idiopathic pulmonary fibrosis
Athol U. Wells
European Respiratory Review Jun 2013, 22 (128) 158-162; DOI: 10.1183/09059180.00001213

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Managing diagnostic procedures in idiopathic pulmonary fibrosis
Athol U. Wells
European Respiratory Review Jun 2013, 22 (128) 158-162; DOI: 10.1183/09059180.00001213
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