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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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Article Information

vol. 23 no. 133 308-319
DOI 
https://doi.org/10.1183/09059180.00004914
PubMed 
25176967

Published By 
European Respiratory Society
Print ISSN 
0905-9180
Online ISSN 
1600-0617
History 
  • Received June 10, 2014
  • Accepted June 15, 2014
  • Published online August 31, 2014.

Copyright & Usage 
©ERS 2014 ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

Author Information

  1. Wim A. Wuyts1,
  2. Alberto Cavazza2,
  3. Giulio Rossi3,
  4. Francesco Bonella4,
  5. Nicola Sverzellati5 and
  6. Paolo Spagnolo6⇑
  1. 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.
  1. Paolo Spagnolo, Medical University Clinic, Canton Hospital Baselland, and University of Basel, Rheinstrasse 26, 4410 Liestal, Switzerland. E-mail: paolo.spagnolo{at}ksbl.ch
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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
    • Concluding remarks
    • Acknowledgments
    • Footnotes
    • References
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Subjects

  • Interstitial and orphan lung disease
  • Lung biology and experimental studies
  • Mechanisms of lung disease
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