Skip to main content
SpringerLink
Log in

Lungenfibrose

Pulmonary fibrosis

  • Schwerpunkt
  • Published:
Der Internist Aims and scope Submit manuscript

Zusammenfassung

Die Prävalenz der Lungenfibrose nimmt stetig zu. Diese ist definiert über die vermehrte Ansammlung von Bindegewebe im Interstitium, das sich diffus ausbreitet und zu einer Zerstörung der normalen Lungenarchitektur führt. Alte Menschen sind deutlich häufiger betroffen als junge Menschen. Etwa die Hälfte aller fibrotischen Lungenveränderungen wird durch zugrunde liegende rheumatologische Erkrankungen hervorgerufen. Nach der im Jahre 1998 erschienen Klassifikation werden die Lungenfibrosen anhand pathologischer Merkmale nun in 6 verschiedene Typen unterschieden. Nach wie vor gibt es keinen effektiven Therapieansatz, der zu einer Auflösung fibrotischer Veränderungen in der Lunge führt. Immunsuppressive Therapieschemata haben sich jedoch bewährt, insbesondere wenn die Lungenfibrose durch inflammatorische Prozesse getriggert wird.

Abstract

Many, different diseases can result in pulmonary fibrosis and its prevalence is continuously increasing. Pulmonary fibrosis is defined by a diffuse accumulation of connective tissue in the interstitial space resulting in destruction of lung parenchyma. Older individuals are more often affected by this disease than younger. Approximately one half of all patients with pulmonary fibrosis suffer from rheumatic diseases. The classification of pulmonary fibrosis was revised 1998. Now 6 different types of interstitial pneumonia according to 6 different pathologic patterns are newly defined. Still, there is no efficient treatment known, which resolves fibrotic lung remodeling. However, immunosuppressive treatment strategies are established in pulmonary fibrosis evoked by inflammatory processes.

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
Abb. 2
Abb. 3

Literatur

  1. Alalawi R, Whelan T, Bajwa RS, Hodges TN (2005) Lung transplantation and interstitial lung disease. Curr Opin Pulm Med 11: 461–466

    Article  PubMed  Google Scholar 

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

    Google Scholar 

  3. American Thoracic Society, European Respiratory Society (2002) International multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 165: 277–304

    Google Scholar 

  4. Antoniou KM, Margaritopoulos G, Economidou F, Siafakas NM (2009) Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement. Eur Respir J 33: 882–896

    Article  CAS  PubMed  Google Scholar 

  5. Demedts M, Behr J, Buhl R, Costabel U et al.; IFIGENIA Study Group (2005) High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 353: 2229–2242

    Article  CAS  PubMed  Google Scholar 

  6. Hervier B, Pagnoux C, Agard C, Haroche J et al. (2009) Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature. Ann Rheum Dis 68: 404–407

    Article  CAS  PubMed  Google Scholar 

  7. Hunzelmann N, Genth E, Krieg T, Lehmacher W et al. (2008) The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology 47: 1185–1192

    Article  CAS  PubMed  Google Scholar 

  8. Ohshimo S, Bonella F, Cui A, Beume M et al. (2009) Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 179: 1043–1047

    Article  PubMed  Google Scholar 

  9. Olson AL, Swigris JJ, Lezotte DC, Norris JM et al. (2007) Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med 176: 277–284

    Article  PubMed  Google Scholar 

  10. Prasse A, Germann M, Müller-Quernheim J (2005) Lungenbeteiligung bei Sklerodermie. Pneumologie 5: 299–307

    Google Scholar 

  11. Prasse A, Müller-Quernheim J (2006) Therapie der idiopathischen interstitiellen Pneumonien. Steroide und was noch? Internist 47: 1258–1262

    Article  CAS  PubMed  Google Scholar 

  12. Ryu JH (2001) Smoking-related interstitial lung diseases: a concise review. Eur Respir J 17: 122–132

    Article  CAS  PubMed  Google Scholar 

  13. Schlesinger C, Koss MN (2005) The organizing pneumonias: an update and review. Curr Opin Pulm Med 11: 422–430

    Article  PubMed  Google Scholar 

  14. Schweisfurth H, Kieslich C, Satake N, Loddenkemper R et al. (2003) Wie werden interstitielle Lungenerkrankungen in Deutschland diagnostiziert? Ergebnisse des wissenschaftlichen Registers zur Erforschung von interstitiellen Lungenerkrankungen („Fibroseregister“) der WATL. Pneumologie 57: 373–382

    Article  CAS  PubMed  Google Scholar 

  15. Tashkin DP, Elashoff R, Clements PJ, Scleroderma Lung Study Research Group (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354: 2655–2666

    Article  CAS  PubMed  Google Scholar 

  16. Turesson C, Jacobsson LTH (2004) Epidemiology of extra-articular manifestations of rheumatoid arthritis. Scand J Rheumatol 33: 65–72

    Article  CAS  PubMed  Google Scholar 

Download references

Interessenkonflikt

Die korrespondierende Autorin gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Abteilung für Pneumologie, Universitätsklinikum Freiburg, Killianstraße 5, 79106, Freiburg, Deutschland

    A. Prasse & J. Müller-Quernheim

  2. Poliklinik für Rheumatologie, Vaskulitiszentrum UKSH & Klinikum Bad Bramstedt, Universität zu Lübeck, Lübeck, Deutschland

    J.U. Holle

Authors
  1. A. Prasse
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J.U. Holle
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. Müller-Quernheim
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to A. Prasse.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Prasse, A., Holle, J. & Müller-Quernheim, J. Lungenfibrose. Internist 51, 6–13 (2010). https://doi.org/10.1007/s00108-009-2406-y

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00108-009-2406-y

Schlüsselwörter

Keywords

Search

Navigation