Skip to main content
SpringerLink
Log in

Therapie der idiopathischen Lungenfibrose

Therapy of idiopathic pulmonary fibrosis

  • Leitthema
  • Published:
Der Pneumologe Aims and scope

Zusammenfassung

Für die idiopathische Lungenfibrose (IPF) stehen pharmakologische und nichtpharmakologische Behandlungsansätze zur Verfügung. Die bisher weit verbreitete Kombinationstherapie aus Prednison, Azathioprin und N-Acetylcystein erwies sich in einer neuen Studie als unwirksam, ja sogar potentiell nachteilig für Patienten mit idiopathischer Lungenfibrose. Auch der pathophysiologisch gut begründete Ansatz der Antikoagulation stellte sich in einer randomisiert-kontrollierten Studie als eher schädlich heraus. Beide Therapien können somit bei gesicherter IPF-Diagnose nicht mehr empfohlen werden. Pirfenidon führt zu einer 30%igen Reduktion der Krankheitsprogression und ist derzeit die einzige zugelassene Therapie für IPF-Patienten mit mildem bis moderatem Schweregrad. Hochdosiertes N-Acetylcystein stellt als antioxidativer Therapieansatz eine Alternative dar, allerdings im „Off-Label“-Verfahren, da hierfür keine Zulassung besteht. Angesichts der begrenzten Wirksamkeit der verfügbaren Therapien sollten IPF-Patienten möglichst in klinische Studien eingeschlossen werden. Seitens der nichtpharmakologischen Therapieansätze stellen die Sauerstofflangzeittherapie und die pulmonale Rehabilitation Grundpfeiler dar. Für Patienten im Alterssegment unter 60 (bis max. 65) Jahren ist die Lungentransplantation die derzeit erfolgversprechendste Behandlungsoption.

Abstract

For patients with idiopathic pulmonary fibrosis (IPF) pharmacological and non-pharmacological therapies are available. The formerly widely used combination of prednisone, azathioprine and N-acetylcysteine has proven to be ineffective and even potentially harmful in a recent randomized controlled treatment trial and is no longer recommended for patients with IPF. Similarly, the pathophysiologically appealing anticoagulation treatment was found to be ineffective and potentially harmful in IPF. For pirfenidone a 30 % reduction in disease progression was demonstrated, consequently, pirfenidone is the only approved therapy in the European Union and Japan for mild to moderate IPF and is the mainstay of current pharmacotherapy. Alternatively, antioxidative therapy with high-dose oral N-acetylcysteine can be used “off-label”. In view of the limited treatment effects of currently available therapies inclusion of IPF patients in treatment trials is a viable option. Non-pharmacological therapies include long-term oxygen therapy and pulmonary rehabilitation. For younger patients (age < 60–65 years) lung transplantation is the treatment of choice.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1

Literatur

  1. Raghu G, Collard HR, Egan JJ et al (2011) ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 183:788–824

    Article  PubMed  Google Scholar 

  2. King TE Jr, Pardo A, Selman M (2011) Idiopathic pulmonary fibrosis. Lancet 378:1949–1961

    Article  PubMed  Google Scholar 

  3. Behr J (2012) Idiopathische Lungenfibrose – moderne, leitliniengerechte Diagnostik und innovative Therapien. Dtsch Med Wochenschr 137:601–604

    Article  PubMed  CAS  Google Scholar 

  4. Fernandez IE, Eickelberg O (2012) New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis. Lancet 380:680–688

    Article  PubMed  CAS  Google Scholar 

  5. Behr J (2012) Approach to the diagnosis of interstitial lung disease. Clin Chest Med 33:1–10

    Article  PubMed  Google Scholar 

  6. American Thoracic Society, European Respiratory Society (2000) American Thoracic Society/European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med 161:646–664

    Google Scholar 

  7. Cantin AM, Hubbard RC, Crystal RG (1989) Glutathione deficiency in the epithelial lining fluid of the lower respiratory tract in idiopathic pulmonary fibrosis. Am Rev Respir Dis 139:370–372

    Article  PubMed  CAS  Google Scholar 

  8. Behr J, Maier K, Degenkolb B et al (1997) Antioxidative and clinical effects of high-dose N-acetylcysteine in fibrosing alveolitis. Adjunctive therapy to maintenance immunosuppression. Am J Respir Crit Care Med 156:1897–1901

    PubMed  CAS  Google Scholar 

  9. Demedts M, Behr J, Buhl R et al (2005) High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 353:2229–2242

    Article  PubMed  CAS  Google Scholar 

  10. Hunninghake GW (2005) Antioxidant therapy for idiopathic pulmonary fibrosis. N Engl J Med 353:2285–2287

    Article  PubMed  CAS  Google Scholar 

  11. Wells AU (2006) Antioxidant therapy in idiopathic pulmonary fibrosis: hope is kindled. Eur Respir J 27:664–666

    Article  PubMed  CAS  Google Scholar 

  12. Behr J, Demedts M, Buhl R et al (2009) Lung function in idiopathic pulmonary fibrosis – extended analyses of the IFIGENIA trial. Respir Res 10:101 (PMID: 19860915)

    Google Scholar 

  13. The Idiopathic Pulmonary Fibrosis Clinical Research Network (2012) Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 366:1968–1977

    Article  Google Scholar 

  14. Behr J, Costabel U, Worth H (2011) Stellungnahme der DGP zur Pressemitteilung des National Heart, Lung, and Blood Institute zur Panther-Studie bei IPF-Patienten vom 21.10.2011. Pneumologie 65:724–725

    Article  PubMed  CAS  Google Scholar 

  15. Wells AU, Behr J, Costabel U et al (2012) Triple therapy in idiopathic pulmonary fibrosis: an alarming press release. Eur Respir J 39:805–806

    Article  PubMed  CAS  Google Scholar 

  16. Raghu G, Johnson WC, Lockhart D, Mageto Y (1999) Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study. Am J Respir Crit Care Med 159:1061–1069

    PubMed  CAS  Google Scholar 

  17. Azuma A, Nukiwa T, Tsuboi E et al (2005) Double-blind, placebo controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 171:1040–1047

    Article  PubMed  Google Scholar 

  18. Taniguchi H, Ebina M, Kondoh Y et al (2010) Pirfenidone clinical study group in Japan. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 35:821–829

    Article  PubMed  CAS  Google Scholar 

  19. Noble PW, Albera C, Bradford WZ et al (2011) Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 377:1760–1769

    Article  PubMed  CAS  Google Scholar 

  20. Spagnolo P, Del Giovane C, Luppi F et al (2010) Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 9:CD003134

    PubMed  Google Scholar 

  21. Magnussen H, Kirsten AM, Köhler D et al (2008) Leitlinien zur Langzeitsauerstofftherapie. Pneumologie 62:748–756

    Article  PubMed  CAS  Google Scholar 

  22. Douglas WW, Ryu JH, Schroeder DR (2000) Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med 161:1172–1178

    PubMed  CAS  Google Scholar 

  23. Huppmann P, Sczepanski B, Boensch M et al (2012) Effects of in-patient pulmonary rehabilitation in a specialized centre in patients with interstitial lung disease. Eur Respir J [Epub ahead of print]

  24. Thabut G, Christie JD, Ravaud P et al (2009) Survival after bilateral versus single-lung transplantation for idiopathic pulmonary fibrosis. Ann Intern Med 151:767–774

    PubMed  Google Scholar 

  25. Bois RM du, Weycker D, Albera C et al (2011) Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 184:459–466

    Article  PubMed  Google Scholar 

  26. Bois RM du, Weycker D, Albera C et al (2011) Six-minute-walk test in idiopathic pulmonary fibrosis: test validation and minimal clinically important difference. Am J Respir Crit Care Med 183:1231–1237

    Article  PubMed  Google Scholar 

  27. Bois RM1 du, Weycker D, Albera C et al (2011) Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med 184:1382–1389

    Article  PubMed  Google Scholar 

  28. Hoeper MM, Andreas S, Bastian A et al (2011) Pulmonary hypertension due to chronic lung disease: updated recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 152:45–53

    Article  Google Scholar 

  29. Richeldi L, Costabel U, Selman M et al (2011) Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 365:1079–1087

    Article  PubMed  CAS  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: Honorare für Vorträge und/oder Beratertätigkeit für Actelion, Allmiral, Bayer, Boehringer, Gilead, GSK, InterMune, Lilly, Novartis, Nycomed, Pfizer, Takeda. Forschungsförderung von Actelion, BMBF, DFG, InterMune, WATL.

Author information

Authors and Affiliations

  1. Asklepios Fachkliniken München Gauting, Medizinische Klinik und Poliklinik V, Comprehensive Pneumology CenterKlinikum der Ludwig-Maximilian-Universität München, , Marchioninistr. 15, 81377, München, Deutschland

    J. Behr

Authors
  1. J. Behr
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J. Behr.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Behr, J. Therapie der idiopathischen Lungenfibrose. Pneumologe 10, 98–104 (2013). https://doi.org/10.1007/s10405-012-0607-x

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10405-012-0607-x

Schlüsselwörter

Keywords

Search

Navigation