Skip to main content
SpringerLink
Log in

Interstitielle Lungenerkrankungen und pulmonale Hypertonie

Interstitial lung diseases and pulmonary hypertension

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

Zusammenfassung

Die pulmonale Hypertonie im Rahmen interstitieller Lungenerkrankungen (ILE) hat eine hohe Prävalenz von 30–40%. Sie wird häufig nicht erkannt und kann auch bereits ohne fortgeschrittene Lungenfunktionseinschränkung oder Hypoxie auftreten. Die pulmonale Hypertonie verschlechtert die Prognose einer ILE deutlich. Es gibt eine Reihe gemeinsamer pathogenetischer Mechanismen zwischen parenchymalem und vaskulärem Umbau (Remodeling) wie oxidativer Stress, Zytokinfreisetzung und das Endothelinsystem. Daneben spielen die hypoxische Vasokonstriktion und Destruktion von Gefäßen bei progredienter Fibrosierung eine wesentliche Rolle. Wegen der oft unspezifischen oder fehlenden klinischen Symptomatik helfen Echokardiographie, radiologische Verfahren und Laborparameter wie das NT-proBNP bei der Diagnosestellung, wobei die definitive Diagnose einer pulmonalen Hypertonie nach wie vor durch die Rechtsherzkatheteruntersuchung gestellt wird. Die Therapie der pulmonalen Hypertonie bei ILE ist limitiert und besteht aus der Therapie der Grundkrankheit sowie supportiven Maßnahmen. Neue vasodilatierende Medikamente könnten zukünftig zur Verbesserung der Prognose beitragen. Die Lungen- bzw. Herz-Lungen-Transplantation stellt eine Therapieoption im Endstadium dar.

Abstract

The prevalence of pulmonary hypertension in interstitial lung disease (ILD) is high (30–40%). However, diagnosis of pulmonary hypertension in ILD is often delayed. Pulmonary hypertension can occur in the absence of advanced pulmonary dysfunction or severe hypoxia and is associated with a worse prognosis. A number of pathogenic mechanisms such as oxidative stress, cytokines, and the endothelin system have been implicated in remodeling of both the lung parenchyma and the vessels. In addition, hypoxic vasoconstriction, vascular destruction and progressive fibrosis play an important role. Since clinical signs are often non-specific echocardiography, radiology and laboratory parameters such as NT-proBNP may be helpful. However, the definitive diagnosis of pulmonary hypertension is still confirmed by right heart catheterization. Treatment options of pulmonary hypertension in ILD are limited to the treatment of the underlying diseases. Newer vasodilating drugs may improve the prognosis but have first to be evaluated in clinical trials. Lung or lung and heart transplantation is the therapeutic option in end stage disease.

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) American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. General principles and recommendations. Am J Respir Crit Care Med 165: 277–304

    Google Scholar 

  2. Arcasoy S, Christie J, Ferrari V et al. (2003) Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 167: 735–740

    Article  PubMed  Google Scholar 

  3. Barst RJ, McGoon M, Tobicki A et al. (2004) Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 43 (Suppl): 40S–47S

    Article  PubMed  Google Scholar 

  4. Behr J, Ryu JH (2008) Pulmonary hypertension in interstitial lung disease. Eur Respir J 31: 1357–1367

    Article  PubMed  CAS  Google Scholar 

  5. Budhiraja R, Tuder RM, Hassoun PM (2004) Endothelial dysfunction in pulmonary hypertension. Circulation 109: 159–165

    Article  PubMed  Google Scholar 

  6. Chang B, Wigley FM, White B, Wise RA (2003) Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 30: 2398–2405

    PubMed  Google Scholar 

  7. Evgenov OV, Pacher P, Schmidt PM et al. (2006) NO-independent stimulators and activators of soluble guanylate cyclase: discovery and therapeutic potential. Nat Rev Drug Discov 5: 755–768

    Article  PubMed  CAS  Google Scholar 

  8. Fartoukh M, Humbert M, Capron F et al. (2000) Sever pulmonary hypertension in histiocytosis X. Am J Respir Crit Care Med 161: 216–223

    PubMed  CAS  Google Scholar 

  9. Foley RJ, Metersky ML (2008) Successful treatment of sarcoidosis-associated pulmonary hypertension with bosentan. Respiration 75: 211–214

    Article  PubMed  CAS  Google Scholar 

  10. Ghofrani HA, Wiedemann R, Rose F et al. (2002) Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomized controlled trial. Lancet 360: 895–900

    Article  PubMed  CAS  Google Scholar 

  11. Giad A, Michel RP, Stewart DJ et al. (1993) Expression of endothelin-1 in lungs of patients with cryptogenic fibrosing alveolitis. Lancet 341: 1550–1554

    Article  Google Scholar 

  12. Hamada K, Nagai S, Tanaka S et al. (2007) Significance of pulmonary arterial pressure and diffusion of the lung as prognosticators in patients with idiopathic pulmonary fibrosis. Chest 131: 650–656

    Article  PubMed  Google Scholar 

  13. Handa T, Nagai S, Mikki S et al. (2006) Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 129: 1246–1252

    Article  PubMed  Google Scholar 

  14. Hopkins N, McLoughlin P (2002) The structural basis of pulmonary hypertension in chronic lung disease: remodelling, rarefaction or angiogenesis. J Anat 201: 335–348

    Article  PubMed  Google Scholar 

  15. King TE, Behr J, Brown KK et al. (2008) BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 177: 75–81

    Article  PubMed  CAS  Google Scholar 

  16. Kinnula V, Fattman C, Tan R, Oury T (2008) Oxidative stress in pulmonary fibrosis: a possible role for redox modulatory therapy. Am J Respir Crit Care Med 177: 219–226

    Google Scholar 

  17. Kubo H, Nakayama K, Yanai M et al. (2005) Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 128: 1475–1482

    Article  PubMed  CAS  Google Scholar 

  18. Lettieri C, Nathan S, Barnett S et al. (2006) Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 129: 746–752

    Article  PubMed  Google Scholar 

  19. Leuchte HH, Baumgartner RA, Nounou ME et al. (2006) Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care Med 173: 744–750

    Article  PubMed  CAS  Google Scholar 

  20. Leuchte HH, Neurohr C, Baumgartner R et al. (2004) Brain natriuretic peptide and exercise capacity in lung fibrosis and pulmonary hypertension. Am J Respir Crit Care Med 170: 360–365

    Article  PubMed  Google Scholar 

  21. Nadrous HF, Pellikka PA, Krowka MJ et al. (2005) Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 128: 2392–2399

    Article  Google Scholar 

  22. Nunes H, Humbert M, Capron F et al. (2006) Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 61: 68–74

    Article  PubMed  CAS  Google Scholar 

  23. Olschewski H, Ghofrani HA, Walmrath D et al. (1999) Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med 160: 600–607

    PubMed  CAS  Google Scholar 

  24. Park SH, Saleh D, Giaid A, Michel RP (1997) Increased endothelin-1 in bleomycin-induced pulmonary fibrosis and the effect of an endothelin receptor antagonist. Am J Respir Crit Care Med 156: 600–608

    PubMed  CAS  Google Scholar 

  25. Rodman DM, Lindenfeld J (1990) Successful treatment of sarcoidosis-associated pulmonary hypertension with corticosteroids. Chest 97: 500–502

    Article  PubMed  CAS  Google Scholar 

  26. Ryu JH, Krowka MJ, Pellikka PA et al. (2007) Pulmonary hypertension in patients with interstitial lung diseases. Mayo Clin Proc 82: 342–350

    Article  PubMed  Google Scholar 

  27. Sanchez O, Sitbon O, Jais X et al. (2006) Immunosuppressive therapy in connective tissue disease-associated pulmonary arterial hypertension. Chest 130: 182–189

    Article  PubMed  CAS  Google Scholar 

  28. Sanz J, Kuschnir P, Rius T et al. (2007) Pulmonary arterial hypertension: noninvasive detection with phase-contrast MR imaging. Radiology 243: 70–79

    Article  PubMed  Google Scholar 

  29. Strange C, Highland KB (2005) Pulmonary hypertension in interstitial lung disease. Curr Opin Pulm Med 9: 426–430

    Google Scholar 

  30. Trulock E, Edwards L, Taylor D et al. (2006) Registry oft the International Society for Heart and Lung Transplantation: twenty-third official adult lung and heart-lung transplantation report 2006. J Heart Lung Transplant 25: 880–892

    Article  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin:

Reisekosten, Kongressteilnahme: Actelion, Encysive, Bayer Health Care.

Author information

Authors and Affiliations

  1. Medizinische Klinik, Forschungszentrum Borstel, Parkallee 35, 23845, Borstel, Deutschland

    H.P. Hauber

Authors
  1. H.P. Hauber
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to H.P. Hauber.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hauber, H. Interstitielle Lungenerkrankungen und pulmonale Hypertonie. Internist 50, 1072–1079 (2009). https://doi.org/10.1007/s00108-009-2339-5

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00108-009-2339-5

Schlüsselwörter

Keywords

Search

Navigation