Skip to main content
SpringerLink
Log in

Differenzielle Therapie bei Lungenfibrosen

Differential therapy of pulmonary fibrosis

  • Individualisierte Therapie – ein Paradigmenwechsel?
  • Published:
Der Internist Aims and scope Submit manuscript

Zusammenfassung

Der Begriff Lungenfibrose stellt kein ätiologisch einheitliches Erkrankungsbild dar, sondern fasst eine Vielzahl diffus parenchymatöser Lungenerkrankungen zusammen, die in einen progressiven, fibrotischen Umbau der Lunge einmünden können. Voraussetzung für jede Therapie ist daher zunächst die sichere diagnostische Einordnung des vorliegenden Krankheitsbildes. Profiling von Gewebe oder bronchoalveolärer Lavage im Sinne von Transkriptom- oder Proteomanalysen könnten zukünftig einen Beitrag zur Diagnosefindung leisten, befinden sich aber derzeit noch in der Entwicklung. Prinzipiell kann man die diffus parenchymatösen Lungenerkrankungen in solche mit primär entzündlichem Triggermechanismus und in der Regel guter Ansprache auf eine immunsuppressive Therapie (z. B. Sarkoidose) und solche mit nicht entzündlichem Triggermechanismus und fehlender Ansprache auf einen solchen Therapieansatz (z. B. idiopathische pulmonale Fibrose) einteilen. Bei letztgenannter Gruppe sind die Therapiemöglichkeiten derzeit noch unbefriedigend und beschränken sich auf die Teilnahme an Studien.

Abstract

Pulmonary fibrosis is the final outcome of a numerous and heterogeneous group of pulmonary disorders grouped together under the family of diffuse parenchymal lung diseases. Safe identification of the underlying condition is difficult but is the prerequisite for any therapeutic attempt. In principle, diffuse parenchymal lung diseases may be divided into those forms being triggered by an initial inflammatory process (e. g. sarcoidosis, hypersensitivity pneumonitis), and those being most likely triggered by epithelial injury (idiopathic pulmonary fibrosis). Steroids and immunosuppressants do have their role in treatment of the former group, although the efficacy on long-term outcome is not entirely clear. In contrast, steroids and immunosuppressants are only rarely helpful in the latter condition. Novel therapeutic strategies for the treatment of idiopathic pulmonary fibrosis are currently under preclinical or clinical assessment and include antioxidative agents and agents that block alveolar coagulation or different growth factors.

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. 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

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  3. 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 

  4. Burrell R, Rylander R (1981) A critical review of the role of precipitins in hypersensitivity pneumonitis. Eur J Respir Dis 62: 332–343

    PubMed  Google Scholar 

  5. Buschman DL, Gamsu G, Waldron JA et al. (1992) Chronic hypersensitivity pneumonitis: use of CT in diagnosis. Am J Roentgenol 159: 957–960

    Google Scholar 

  6. Costabel U (1997) CD4/CD8 ratios in bronchoalveolar lavage fluid: of value for diagnosing sarcoidosis? Eur Respir J 10: 2699–2700

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  8. du Bois RM, Greenhalgh PM, Southcott AM et al. (1999) Randomized trial of inhaled fluticasone propionate in chronic stable pulmonary sarcoidosis: a pilot study. Eur Respir J 13: 1345–1350

    Article  PubMed  Google Scholar 

  9. Gibson GJ, Prescott RJ, Muers MF et al. (1996) British Thoracic Society Sarcoidosis study: effects of long term corticosteroid treatment. Thorax 51: 238–247

    PubMed  Google Scholar 

  10. Gilman MJ, Wang KP (1980) Transbronchial lung biopsy in sarcoidosis. An approach to determine the optimal number of biopsies. Am Rev Respir Dis 122: 721–724

    PubMed  Google Scholar 

  11. Hendrick DJ, Marshall R, Faux JA, Krall JM (1981) Protective value of dust respirators in extrinsic allergic alveolitis: clinical assessment using inhalation provocation tests. Thorax 36: 917–921

    PubMed  Google Scholar 

  12. Hunninghake GW, Gilbert S, Pueringer R et al. (1994) Outcome of the treatment for sarcoidosis. Am J Respir Crit Care Med 149: 893–898

    PubMed  Google Scholar 

  13. Hunninghake GW, Costabel U, Ando M et al. (1999) ATS/ERS/WASOG statement on sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 16: 149–173

    PubMed  Google Scholar 

  14. Keller RH, Swartz S, Schlueter DP et al. (1984) Immunoregulation in hypersensitivity pneumonitis. Phenotypic and functional studies of bronchoalveolar lavage lymphocytes. Am Rev Respir Dis 130: 766–771

    PubMed  Google Scholar 

  15. Kokkarinen JI, Tukjainen HO, Terho EO (1992) Effect of corticosteroid treatment on the recovery of pulmonary function in farmer’s lung. Am Rev Respir Dis 145: 3–5

    PubMed  Google Scholar 

  16. Lower EE, Baughman RP (1995) Prolonged use of methotrexate for sarcoidosis. Arch Intern Med 155: 846–851

    Article  PubMed  Google Scholar 

  17. Pacheco Y, Marechal C, Marechal F et al. (1985) Azathioprine treatment of chronic pulmonary sarcoidosis. Sarcoidosis 2: 107–113

    PubMed  Google Scholar 

  18. Pardo A, Selman M (2002) Idiopathic pulmonary fibrosis: new insights in its pathogenesis. Int J Biochem Cell Biol 34: 1534–1538

    Article  PubMed  Google Scholar 

  19. Patterson R, Wang JL, Fink JN et al. (1979) IgA and IgG antibody activities of serum and bronchoalveolar fluid from symptomatic and asymptomatic pigeon breeders. Am Rev Respir Dis 120: 1113–1118

    PubMed  Google Scholar 

  20. Raghu G, Brown KK, Bradford WZ et al.; Idiopathic Pulmonary Fibrosis Study Group (2004) A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med 350: 125–133

    Article  PubMed  Google Scholar 

  21. Richerson HB, Bernstein IL, Fink JN et al. (1989) Guidelines for the clinical evaluation of hypersensitivity pneumonitis. Report of the Subcommittee on Hypersensitivity Pneumonitis. J Allergy Clin Immunol 84: 839–844

    Article  PubMed  Google Scholar 

  22. Selroos O, Lofroos AB, Pietinalho A et al. (1994) Inhaled budesonide for maintenance treatment of pulmonary sarcoidosis. Sarcoidosis 11: 126–131

    PubMed  Google Scholar 

  23. Selman M, King TE, Pardo A (2001) Idiopathic pulmonary fibrosis: Prevaling and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med 134: 136–151

    PubMed  Google Scholar 

  24. Selman M, Pardo A, Barrera L et al. (2006) Gene expression profiles distinguish idiopathic pulmonary fibrosis from hypersensitivity pneumonitis. Am J Respir Crit Care Med 173: 188–198

    Article  PubMed  Google Scholar 

  25. Sime PJ, Xing Z, Graham FL, Gauldie J (1997) Adenoviral-mediated gene transfer of active TGF-beta 1 induces prolonged aggressive fibrosis in rat lung. J Clin Invest 100: 768–776

    PubMed  Google Scholar 

  26. Thannickal VJ, Toews GB, White ES et al. (2004) Mechanisms of pulmonary fibrosis. Annu Rev Med 55: 395–417

    Article  PubMed  Google Scholar 

  27. Wells A (1998) High resolution computed tomography in sarcoidosis: a clinical perspective. Sarcoidosis Vasc Diffuse Lung Dis 15: 140–146

    PubMed  Google Scholar 

  28. Winterbauer RH, Lammert J, Selland M et al. (1993) Bronchoalveolar lavage cell populations in the diagnosis of sarcoidosis. Chest 104: 352–361

    PubMed  Google Scholar 

  29. Ziesche R, Hofbauer E, Wittmann K et al. (1999) A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis. N Engl J Med 341: 1264–1269

    Article  PubMed  Google Scholar 

Download references

Interessenkonflikt

Es besteht kein Interessenkonflikt. Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen. Die Präsentation des Themas ist unabhängig und die Darstellung der Inhalte produktneutral.

Author information

Authors and Affiliations

  1. Medizinische Klinik II und Poliklinik, Justus-Liebig-Universität, Klinikstraße 36, 35392, Giessen

    P. Markart, W. Seeger &  A. Günther

Authors
  1. P. Markart
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. W. Seeger
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A. Günther
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to A. Günther.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Markart, P., Seeger, W. & Günther, A. Differenzielle Therapie bei Lungenfibrosen. Internist 47 (Suppl 1), S26–S32 (2006). https://doi.org/10.1007/s00108-006-1641-8

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00108-006-1641-8

Schlüsselwörter

Keywords

Search

Navigation