Severe idiopathic pulmonary fibrosis

A clinical approach

R. Lipsi, D. Mazzola, A. Caminati, D. Elia, C. Lonati, S. Harari

Research output: Contribution to journalArticle

Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease associated with a high mortality rate. Novel antifibrotic therapies have been recently demonstrated to slow disease progression and improve survival. However, the management of IPF remains a difficult challenge, since lung complications can still occur, particularly in patients with advanced-stage disease. This paper highlights the most common complications and difficult tasks related to severe IPF such as acute exacerbation of the disease, development of lung cancer, rapid disease progression, and indication for lung transplantation.

Original languageEnglish
JournalEuropean Journal of Internal Medicine
DOIs
Publication statusAccepted/In press - Jan 1 2018
Externally publishedYes

Fingerprint

Idiopathic Pulmonary Fibrosis
Disease Progression
Lung Transplantation
Acute Disease
Lung Neoplasms
Lung
Survival
Mortality
Therapeutics

Keywords

  • Acute exacerbation of IPF
  • Lung cancer
  • Lung transplantation
  • Nintedanib
  • Palliation
  • Pirfenidone

ASJC Scopus subject areas

  • Internal Medicine

Cite this

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AU - Mazzola, D.

AU - Caminati, A.

AU - Elia, D.

AU - Lonati, C.

AU - Harari, S.

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AB - Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease associated with a high mortality rate. Novel antifibrotic therapies have been recently demonstrated to slow disease progression and improve survival. However, the management of IPF remains a difficult challenge, since lung complications can still occur, particularly in patients with advanced-stage disease. This paper highlights the most common complications and difficult tasks related to severe IPF such as acute exacerbation of the disease, development of lung cancer, rapid disease progression, and indication for lung transplantation.

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KW - Lung transplantation

KW - Nintedanib

KW - Palliation

KW - Pirfenidone

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