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Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?

Wim A. Wuyts, Alberto Cavazza, Giulio Rossi, Francesco Bonella, Nicola Sverzellati, Paolo Spagnolo
European Respiratory Review 2014 23: 308-319; DOI: 10.1183/09059180.00004914
Wim A. Wuyts
1Unit for Interstitial Lung Diseases, Dept of Pulmonary Medicine, University Hospitals Leuven, Leuven, Belgium. 2Operative Unit of Pathology, S. Maria Nuova Hospital, Reggio Emilia, Italy. 3Section of Pathology, University Hospital of Modena, Modena, Italy. 4Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy. 5Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Germany. 6Medical University Clinic, Canton Hospital Baselland and University of Basel, Liestal, Switzerland.
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Alberto Cavazza
1Unit for Interstitial Lung Diseases, Dept of Pulmonary Medicine, University Hospitals Leuven, Leuven, Belgium. 2Operative Unit of Pathology, S. Maria Nuova Hospital, Reggio Emilia, Italy. 3Section of Pathology, University Hospital of Modena, Modena, Italy. 4Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy. 5Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Germany. 6Medical University Clinic, Canton Hospital Baselland and University of Basel, Liestal, Switzerland.
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Giulio Rossi
1Unit for Interstitial Lung Diseases, Dept of Pulmonary Medicine, University Hospitals Leuven, Leuven, Belgium. 2Operative Unit of Pathology, S. Maria Nuova Hospital, Reggio Emilia, Italy. 3Section of Pathology, University Hospital of Modena, Modena, Italy. 4Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy. 5Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Germany. 6Medical University Clinic, Canton Hospital Baselland and University of Basel, Liestal, Switzerland.
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Francesco Bonella
1Unit for Interstitial Lung Diseases, Dept of Pulmonary Medicine, University Hospitals Leuven, Leuven, Belgium. 2Operative Unit of Pathology, S. Maria Nuova Hospital, Reggio Emilia, Italy. 3Section of Pathology, University Hospital of Modena, Modena, Italy. 4Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy. 5Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Germany. 6Medical University Clinic, Canton Hospital Baselland and University of Basel, Liestal, Switzerland.
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Nicola Sverzellati
1Unit for Interstitial Lung Diseases, Dept of Pulmonary Medicine, University Hospitals Leuven, Leuven, Belgium. 2Operative Unit of Pathology, S. Maria Nuova Hospital, Reggio Emilia, Italy. 3Section of Pathology, University Hospital of Modena, Modena, Italy. 4Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy. 5Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Germany. 6Medical University Clinic, Canton Hospital Baselland and University of Basel, Liestal, Switzerland.
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Paolo Spagnolo
1Unit for Interstitial Lung Diseases, Dept of Pulmonary Medicine, University Hospitals Leuven, Leuven, Belgium. 2Operative Unit of Pathology, S. Maria Nuova Hospital, Reggio Emilia, Italy. 3Section of Pathology, University Hospital of Modena, Modena, Italy. 4Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy. 5Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Germany. 6Medical University Clinic, Canton Hospital Baselland and University of Basel, Liestal, Switzerland.
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  • For correspondence: paolo.spagnolo@ksbl.ch
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  • Figure 1.
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    Figure 1.

    A possible usual interstitial pneumonia pattern. Computed tomography scan showing subpleural and bibasal predominant reticular abnormality without honeycombing. Although this patient was highly likely to have idiopathic pulmonary fibrosis (in the appropriate clinical setting), surgical lung biopsy is needed to confirm the diagnosis.

  • Figure 2.
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    Figure 2.

    a) Open lung biopsy showing fibrotic lung disease with heterogeneous spatial and temporal fibrosis consistent with a usual interstitial pneumonia pattern (haematoxylin and eosin stain, 40×). b) Honeycomb change with enlarged subpleural alveolar spaces with bronchiolar metaplasia, mucus plugs, and mild chronic inflammatory infiltrate (haematoxylin and eosin stain, 40×).

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    Figure 3.

    Chronic hypersensitivity pneumonitis with the computed tomography scan showing extensive reticular and ground-glass opacities. Traction bronchiectasis is also present in the apical segment of the left lower lobe. The left fissure is pulled apart because of the underlying fibrosis.

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    Figure 4.

    Usual interstitial pneumonia pattern in two patients with idiopathic pulmonary fibrosis. a) At low magnification, subpleural/paraseptal scars obscuring the alveolar architecture alternate with normal lung (“patchy fibrosis”). At the top, two areas of honeycombing can be observed (haematoxylin and eosin stain, 20×). b) At higher magnification a fibroblastic focus, consisting of a dome-shaped collection of fibroblasts/myofibroblasts embedded in a myxoid matrix and covered by hyperplastic pneumocytes, is observed. Note the pale grey colour of the fibroblastic focus, contrasting with the pink colour of the underlying scar (haematoxylin and eosin stain, 400×).

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    Figure 5.

    Chronic hypersensitivity pneumonitis with usual interstitial pneumonia pattern. a) At low magnification, bronchiolar damage with bridging fibrosis between bronchiole and pleura are the clues suggesting chronic hypersensitivity pneumonitis (haematoxylin and eosin stain, 20×). b) At higher magnification, a few inconspicuous interstitial granulomas, consisting of isolated giant cells with cholesterol clefts, can be observed (haematoxylin and eosin stain, 200×).

Tables

  • Figures
  • Table 1. Histological clues that, when present in the usual interstitial pneumonia pattern, suggest the possibility of an underlying connective tissue disease (CTD) or chronic hypersensitivity pneumonitis (HP)
    FeatureCTDChronic HP
    Cellular (lymphocytic and/or plasmacellular) interstitial infiltrate++
    Plasma cells++
    Cellular bronchiolitis++
    Centrilobular fibrosis, with or without bridging fibrosis between bronchioles and pleura−+
    Pleuritis+−
    Small interstitial/peribronchiolar granulomas–#+
    Coexistence of more than one pattern in the same biopsy+−/+
    • +: positive; –: negative. #: except in Sjögren’s syndrome, in which small granulomas can be found.

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Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?
Wim A. Wuyts, Alberto Cavazza, Giulio Rossi, Francesco Bonella, Nicola Sverzellati, Paolo Spagnolo
European Respiratory Review Sep 2014, 23 (133) 308-319; DOI: 10.1183/09059180.00004914

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Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?
Wim A. Wuyts, Alberto Cavazza, Giulio Rossi, Francesco Bonella, Nicola Sverzellati, Paolo Spagnolo
European Respiratory Review Sep 2014, 23 (133) 308-319; DOI: 10.1183/09059180.00004914
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  • Article
    • Abstract
    • Clinical case
    • Challenges in diagnosing IPF in clinical practice
    • Delay in diagnosis
    • Excluding known causes of pulmonary fibrosis
    • Determining when a confirmatory SLB is needed
    • Differential diagnosis of UIP pattern
    • UIP pattern on histology: considerations for the clinician
    • Role of BAL in the differential diagnosis
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