Elisabeth Bendstrup

Treatment of idiopathic pulmonary fibrosis: a position paper from a Nordic expert group

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperReviewResearchpeer-review

DOI

  • C. M. Skold, Karolinska Univ Hosp Solna, Karolinska University Hospital, Karolinska Institutet, Lung Allergy Clin
  • ,
  • E. Bendstrup
  • M. Myllarniemi, Univ Helsinki, Helsinki University Central Hospital, University of Helsinki, Skin & Allergy Hosp, Cent Hosp
  • ,
  • G. Gudmundsson, Univ Iceland, Landspitali National University Hospital, University of Iceland, Landspitali Univ Hosp, Dept Resp Med & Sleep, Fac Med
  • ,
  • T. Sjaheim, Natl Hosp Norway, University of Oslo, Oslo Univ Hosp, Dept Resp Med
  • ,
  • O. Hilberg
  • ,
  • A. Altraja, Tartu Univ Hosp, Lung Clin
  • ,
  • R. Kaarteenaho, Kuopio Univ Hosp, University of Eastern Finland
  • ,
  • G. Ferrara, Karolinska Univ Hosp Solna, Karolinska University Hospital, Karolinska Institutet, Lung Allergy Clin

Idiopathic pulmonary fibrosis (IPF) is a fatal progressive lung disease occurring in adults. In the last decade, the results of a number of clinical trials based on the updated disease classification have been published. The registration of pirfenidone and nintedanib, the first two pharmacological treatment options approved for IPF, marks a new chapter in the management of patients with this disease. Other nonpharmacological treatments such as lung transplantation, rehabilitation and palliation have also been shown to be beneficial for these patients. In this review, past and present management is discussed based on a comprehensive literature search. A treatment algorithm is presented based on available evidence and our overall clinical experience. In addition, unmet needs with regard to treatment are highlighted and discussed. We describe the development of various treatment options for IPF from the first consensus to recent guidelines based on evidence from large-scale, multinational, randomized clinical trials, which have led to registration of the first drugs for IPF.

Original languageEnglish
JournalJournal of Internal Medicine
Volume281
Issue2
Pages (from-to)149-166
Number of pages18
ISSN0954-6820
DOIs
Publication statusPublished - Feb 2017

    Research areas

  • idiopathic interstitial pneumonias, lung transplantation, nintedanib, pirfenidone, pulmonary rehabilitation, INTERSTITIAL LUNG-DISEASE, PLACEBO-CONTROLLED TRIAL, FORCED VITAL CAPACITY, GASTROESOPHAGEAL-REFLUX THERAPY, RANDOMIZED CONTROLLED-TRIAL, INHALED N-ACETYLCYSTEINE, GENOME-WIDE ASSOCIATION, DOUBLE-BLIND, INTERNATIONAL SOCIETY, LONG-TERM

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