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Orphan Lung Diseases pp 315–325Cite as

Pulmonary Fibrosis and the Many Faces of UIP

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Abstract

Interstitial lung diseases (ILDs) represent a heterogeneous group of clinical entities among which disease of unknown causes may mimic ILDs due to known causes. Clinical, radiographic and histopathology presentation can largely overlap between different entities and a multidisciplinary approach is proven to be essential in composing the puzzle to reach the most likely clinical diagnosis in each single patients. The definition of specific radiographic (on chest high-resolution computed tomography, HRCT) and histopathology (on lung surgical lung biopsy, SLB) patterns has provided a common terminology in the field of ILDs in the tentative of classifying entities presenting with distinctive features. These patterns have been proposed in the classification of idiopathic interstitial pneumonias (IIPs), and then have been applied to describe ILDs due to secondary known causes, particularly those related to connective-tissue diseases. Among all these patterns, the usual interstitial pneumonia (UIP) pattern has received more emphasis, in particular since it identifies patients with idiopathic pulmonary fibrosis (IPF), as outlined in the most recent evidence-based international guideline. In this document, specific HRCT and SLB criteria for the definition of a definite UIP pattern have been proposed and since then they have been widely used as reference standard both in clinical practice and in the definition of eligibility criteria for randomized clinical trials. However, while a definite UIP pattern can be diagnostic for IPF in the proper clinical context, it is well known that the same pattern can be present in fibrotic lung diseases other than IPF, with important consequences in terms of therapeutic management and prognosis. This chapter will provide an overview on how the UIP pattern is defined, both on radiologist’s view and on pathologist’s view, along with elements that might be helpful in distinguishing an idiopathic UIP pattern from similar appearance in secondary diseases.

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Author information

Authors and Affiliations

  1. Centre for Rare Lung Diseases, University Hospital of Modena, Modena, Italy

    Stefania Cerri

  2. Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, UK

    Giacomo Sgalla & Luca Richeldi

  3. Histopathology Unit, University Hospital of Modena, Modena, Italy

    Giulio Rossi

  4. Radiology Unit, University Hospital of Modena, Modena, Italy

    Giovanni Della Casa

  5. Department of Clinical and Experimental Sciences, University Hospital Southampton, Mailpoint 813, LE75, South Academic Block, Tremona Road, Southampton, SO16 6YD, UK

    Luca Richeldi

Authors
  1. Stefania Cerri
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  2. Giacomo Sgalla
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  3. Giulio Rossi
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  4. Giovanni Della Casa
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  5. Luca Richeldi
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Corresponding author

Correspondence to Luca Richeldi .

Editor information

Editors and Affiliations

  1. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Vincent Cottin

  2. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Jean-Francois Cordier

  3. Respiratory Medicine, University of Southampton, Southampton, United Kingdom

    Luca Richeldi

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© 2015 Springer-Verlag London

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Cerri, S., Sgalla, G., Rossi, G., Casa, G.D., Richeldi, L. (2015). Pulmonary Fibrosis and the Many Faces of UIP. In: Cottin, V., Cordier, JF., Richeldi, L. (eds) Orphan Lung Diseases. Springer, London. https://doi.org/10.1007/978-1-4471-2401-6_21

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  • DOI: https://doi.org/10.1007/978-1-4471-2401-6_21

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  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-2400-9

  • Online ISBN: 978-1-4471-2401-6

  • eBook Packages: MedicineMedicine (R0)

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