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Progressive Fibrosing Interstitial Lung Diseases: A Current Perspective.

Carlo Albera1, Giulia Verri2, Federico Sciarrone1

  • 1Department of Medical Sciences, School of Medicine, University of Turin, SC Pneumologia U, 10124 Turin, Italy.

Biomedicines
|September 28, 2021
PubMed
Summary

Interstitial lung diseases (ILDs) with a progressive fibrosing phenotype benefit from antifibrotic therapies like pirfenidone and nintedanib. This review covers their characteristics, pathogenesis, diagnosis, and optimized treatment strategies.

Keywords:
antifibrotic therapyfibrosisidiopathic pulmonary fibrosisinterstitial lung diseasenintedanibpirfenidoneprogressive fibrosing interstitial lung disease

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Area of Science:

  • Pulmonology
  • Fibrotic Lung Diseases
  • Pharmacology

Background:

  • Interstitial lung diseases (ILDs) are rare, chronic respiratory disorders.
  • Idiopathic pulmonary fibrosis (IPF) is the most common ILD.
  • Fibrosis is increasingly recognized as a key therapeutic target in IPF and other ILDs.

Purpose of the Study:

  • To review current knowledge on progressive fibrosing ILDs.
  • To discuss the clinical characteristics, pathogenesis, and mechanisms of lung fibrosis.
  • To summarize diagnostic approaches and treatments, focusing on antifibrotic optimization.

Main Methods:

  • Narrative review of relevant literature.
  • Discussion of clinical and pathogenetic aspects of fibrotic ILDs.
  • Synthesis of diagnostic and therapeutic strategies.

Main Results:

  • Antifibrotic agents (pirfenidone, nintedanib) are approved and beneficial for IPF.
  • A "progressive fibrosing phenotype" is identified in various ILDs, responding to antifibrotics.
  • Optimizing antifibrotic treatment in clinical practice is crucial.

Conclusions:

  • Progressive fibrosing ILDs share disease behavior with IPF.
  • Antifibrotic therapy is effective in ILDs with a progressive fibrosing phenotype.
  • Further optimization of antifibrotic use is needed for improved patient outcomes.