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Unclassifiable interstitial lung disease: a pathologist's perspective

Kirk D. Jones
European Respiratory Review 2018 27: 170132; DOI: 10.1183/16000617.0132-2017
Kirk D. Jones
Dept of Pathology, University of California San Francisco, San Francisco, CA, USA
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  • FIGURE 1
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    FIGURE 1

    Bronchiolocentric fibrosis. a) This low power view shows fibrosis of peribronchiolar alveolar septa. Scale bar=500 µm. b) A higher power view shows peribronchiolar metaplasia with lining of the bronchiolocentric alveolar septa by ciliated columnar respiratory epithelium. Scale bar=200 µm.

  • FIGURE 2
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    FIGURE 2

    Nonspecific interstitial pneumonia. a) This low power view shows diffuse alveolar septal thickening by moderate interstitial fibrosis. Scale bar=1 mm. b) A higher power view shows the architectural preservation of lung tissue. Scale bar=500 µm.

  • FIGURE 3
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    FIGURE 3

    Usual interstitial pneumonia. a) This low power view shows the heterogeneous pattern of fibrosis with subpleural microscopic honeycombing and centrilobular lack of alveolar inflammation or fibrosis. Scale bar=2 mm. b) A high power view shows a fibroblast focus with typical layered appearance within a loose myxoid stroma. Scale bar=100 µm.

  • FIGURE 4
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    FIGURE 4

    Unclassifiable interstitial fibrosis. This low power view shows patchy fibrosis with subpleural and bronchiolocentric accentuation as well as prominent lymphoid aggregates. Further clinical history for this 68-year-old woman suggested the likely multidisciplinary diagnosis was nitrofurantoin toxicity. Scale bar= 2 mm.

  • FIGURE 5
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    FIGURE 5

    Unclassifiable interstitial fibrosis. This low power view shows patchy fibrosis with focal microscopic honeycombing, airspace enlargement, scattered lymphoid aggregates, and central nonspecific interstitial pneumonia fibrosis-like changes. Further clinical history for this 49-year-old woman with early greying of hair and a sibling with pulmonary fibrosis suggested the likely diagnosis was familial interstitial pulmonary fibrosis. Scale bar=2 mm.

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  • TABLE 1

    Multidisciplinary diagnosis of patients pathologically unclassifiable by surgical lung biopsy

    Cases n
     Number of surgical lung biopsies performed: 849
     Number of non-diagnostic biopsies: 2
     Number with pathologic diagnosis of “fibrosing interstitial pneumonia: unclassifiable”: 41
    Final multidisciplinary diagnosis n
     Unclassifiable: 19
     Idiopathic pulmonary fibrosis: 8
     Chronic hypersensitivity pneumonia: 5
     Autoimmune connective tissue disease: 4
     Smoking-related interstitial lung disease: 2
     Infection (Mycobacterium avium): 1
     Sarcoidosis: 1
     Idiopathic nonspecific interstitial pneumonia: 1
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Vol 27 Issue 147 Table of Contents
European Respiratory Review: 27 (147)
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Unclassifiable interstitial lung disease: a pathologist's perspective
Kirk D. Jones
European Respiratory Review Mar 2018, 27 (147) 170132; DOI: 10.1183/16000617.0132-2017

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Unclassifiable interstitial lung disease: a pathologist's perspective
Kirk D. Jones
European Respiratory Review Mar 2018, 27 (147) 170132; DOI: 10.1183/16000617.0132-2017
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • Surgical lung biopsy: reducing pre-histological barriers to classification
    • Typical patterns of fibrosis
    • Physiological basis of the typical patterns of fibrosis
    • Histological sources of unclassifiable interstitial fibrosis
    • Post-biopsy evaluation: the MDD
    • Conclusions
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

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  • Interstitial and orphan lung disease
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