Hopp til hovedinnhold

Lungefibrose, idiopatisk

Sist revidert:
Sist revidert av:
Definisjon:
Idiopatisk lungefibrose (IPF) er en tilstand med gradvis økende fibrose i lungene av ukjent årsak.
Forekomst:
Relativt sjelden, insidens er beregnet til ca. 14-43 tilfeller per 100.000 personer årlig, økende med alderen, diagnosen stilles oftest i 60-års alderen.
Symptomer:
Hovedsymptomer er kronisk og gradvis økende tungpust og hoste.
Funn:
Kan være hviledyspné, takypné og cyanose, clubbing.
Diagnostikk:
Viktige diagnostiske undersøkelser er CT, spirometri, bronkoskopi og ev. torakoskopi med biopsi.
Behandling:
Nye medikamenter har vist å kunne bremse sykdomsutviklingen noe. Lungetransplantasjon kan være aktuelt.
  • Nasjonal behandlingstjeneste for organtransplantasjon, Oslo universitetssykehus HF, nettsted 
  1. Kreider ME, Natalini JG. Idiopathic pulmonary fibrosis. Best Practice, last updated 26 June 2019. bestpractice.bmj.com  
  2. Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188: 733. pmid:24032382 PubMed  
  3. King Jr TE, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Seminar. Lancet 2011; 378: 1949-61. PubMed  
  4. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017;389(10082):1941-1952. PubMed  
  5. Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176:636-643. PubMed  
  6. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788-824. PubMed  
  7. King TE. Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis. UpToDate, last updated Mar 15, 2021. UpToDate  
  8. Raghu G, Rochwerg B, Zhangh Y, et al . An official ATS/ERS/JRS/ALAT clinical practice guideline: Treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Resp Crit Care Med 2015; 192: e3-e19. doi:10.1164/rccm.201506-1063ST DOI  
  9. King TE. Treatment of idiopathic pulmonary fibrosis. UpToDate, last updated Jan 07, 2020. UpToDate  
  10. Dempsey TM, Sangaralingham LR, Yao X, et al. Clinical Effectiveness of Antifibrotic Medications for Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2019; 200: 168-74. pmid:31150266 PubMed  
  11. Loveman E, Copley VR, Colquitt J, et al. The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation. Health Technol Assess. 2015 Mar;19(20):1-336. PubMed  
  12. Cheng L, Tan B, Yin Y, et al. Short- and long-term effects of pulmonary rehabilitation for idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Clin Rehabil. 2018. PMID: 29843523 PubMed  
  13. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011; 377: 1760-9. PubMed  
  14. King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014. doi:10.1056/NEJMoa1402582 DOI  
  15. Kang J, Han M, Song JW. Antifibrotic treatment improves clinical outcomes in patients with idiopathic pulmonary fibrosis: a propensity score matching analysis. Sci Rep 2020; 10: 15620. pmid:32973215 PubMed  
  16. Wuyts WA, Antoniou KM, Borensztajn K, et al. Combination therapy: the future of management for idiopathic pulmonary fibrosis? Lancet Respir Med. 2014 Nov;2:933-42. doi: 10.1016/S2213-2600(14)70232-2 DOI  
  17. Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011; 365: 1079-87. New England Journal of Medicine  
  18. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonar fibrosis. N Engl J Med 2014. doi:10.1056/NEJMoa1402584 DOI  
  19. Raghu G, Anstrom KJ, King TE, et al. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012; 366: 1968-77. New England Journal of Medicine  
  20. Kolb M, Raghu G, Wells AU, et al. Nintedanib plus Sildenafil in Patients with Idiopathic Pulmonary Fibrosis. N Engl J Med 2018. pmid:30220235 PubMed  
  21. Horton MR, Santopietro V, Mathew L, et al. Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis: a randomized trial. Ann Intern Med 2012; 157: 398-406. Annals of Internal Medicine  
  22. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183: 431-40. PubMed  
  • Ingard Løge, spesialist i allmennmedisin, redaktør NEL

Tidligere fagmedarbeidere

  • Hasse Melbye, spesialist i allmennmedisin, førsteamanuensis ved institutt for samfunnsmedisin, Universitetet i Tromsø