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Introduction

Introduction

Interstitial lung diseases (ILDs) are a large, diverse group of parenchymal lung disorders, mostly classified as rare or seen only infrequently in clinical practice.1-3 Pulmonary fibrosis is an insidious threat across many ILDs, including those originating from connective tissue diseases (CTDs) such as systemic sclerosis and rheumatoid arthritis.3-6 The heterogeneity and unpredictability of ILDs can make pulmonary fibrosis a challenge for physicians to detect, often leading to a delayed diagnosis.4,5,7,8

While ILDs differ, common pathogenic pathways to fibrogenesis are shared.3,9,10

The aim of this atlas is to help clinicians recognise lesions consistent with infiltrative lung disease and characteristic aspects of ILDs.

The CT section, while not exhaustive, illustrates the imaging approach in addressing an ILD:

  • Recognition of the predominant sign
  • Recognition of accessory signs
  • Analysis of lesions distribution in the lung and lobule

The histopathology section presents the diagnostic process for fibrosis, as well as situations that may cause confusion. This atlas is therefore intended to assist clinicians throughout the process of diagnosing ILDs.

Chest CT

Histopathology

COORDINATORS: Prof. Gilbert FERRETTI Radiologist, Hôpital Nord, Grenoble University Hospital
Prof. Françoise THIVOLET-BEJUI Pathologist, Hôpital Louis Pradel, Lyon University Hospital

EDITORIAL COMMITTEE: Prof. Bernard AGUILANIU Pulmonologist, Grenoble
Prof. Vincent COTTIN Pulmonologist, Hôpital Louis Pradel, Lyon University Hospital
Dr Grégoire PREVOT Pulmonologist, Hôpital Larrey, Toulouse University Hospital

Reference

References

  1. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017;4(1):e000212.

  2. Demedts M, Wells AU, Antó JM, et al. Interstitial lung diseases. An epidemiological overview. Eur Respir J Suppl. 2001;32:2s-16s.

  3. Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180076

  4. Raghu G, Nyberg F, Morgan G. The epidemiology of interstitial lung disease and its association with lung cancer. Br J Cancer. 2004;91(suppl 2):S3-S10.

  5. Wijsenbeek M, Kreuter M, Fischer A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019:1–10. DOI: 101080/03007995.2019.1647040.

  6. Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ; IPF Consensus Working Group. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5):1800692.

  7. Gulati M. Diagnostic assessment of patients with interstitial lung disease. Prim Care Respir J. 2011;20(2):120 127.

  8. Greiffo FR, Eickelberg O, Fernandez IE. Systems medicine advances in interstitial lung disease. Eur Respir Rev. 2017;26:170021.

  9. Selman M, King TE, Pardo A, et al. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med. 2001;134(2):136-151.

  10. Bagnato G, Harari S. Cellular interactions in the pathogenesis of interstitial lung diseases. Eur Respir Rev. 2015;24(135):102-114.