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Related Experiment Video

Updated: Apr 20, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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[Current classification of interstitial pulmonary diseases].

J Vogel-Claussen1, A Prasse

  • 1Institut für Diagnostische und Interventionelle Radiologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland, Vogel-Claussen.Jens@mh-hannover.de.

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Summary

The 2013 American Thoracic Society (ATS)/European Respiratory Society (ERS) guidelines update pulmonary fibrosis classification. Key changes include recognizing Nonspecific Interstitial Pneumonia (NSIP) as distinct and introducing Pleuroparenchymal fibroelastosis (PPFE).

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Area of Science:

  • Pulmonology
  • Pathology
  • Radiology

Context:

  • The 2002 classification of idiopathic interstitial pneumonia (IIP), also known as pulmonary fibrosis, has been significantly revised.
  • The 2013 update by the American Thoracic Society (ATS) and European Respiratory Society (ERS) addresses advancements in understanding and diagnosing lung fibrosis.

Purpose:

  • To outline the key points of the 2013 ATS/ERS consensus statement for the classification of pulmonary fibrosis.
  • To highlight revisions and new entities within the updated classification system.

Summary:

  • The revised classification emphasizes an interdisciplinary diagnostic approach involving pulmonology, radiology, and pathology.
  • Nonspecific Interstitial Pneumonia (NSIP) is now recognized as a distinct subtype. Smoking-associated respiratory bronchiolitis with interstitial lung disease (RB-ILD) can be diagnosed non-invasively.
  • Idiopathic pulmonary fibrosis (IPF) heterogeneity is acknowledged, and 'acute exacerbation' is defined for acute attacks (<30 days) in IPF and NSIP. Pleuroparenchymal fibroelastosis (PPFE) is identified as a rare new entity.

Impact:

  • The 2013 update refines the diagnosis and management of various pulmonary fibrosis subtypes.
  • It provides a framework for clinical classification algorithms and highlights the potential of molecular markers for future diagnostic improvements.
  • This revision serves as a supplement to the 2002 classification, reflecting progress and guiding future research in lung fibrosis.