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Der Internist

, Volume 50, Issue 9, pp 1072–1079 | Cite as

Interstitielle Lungenerkrankungen und pulmonale Hypertonie

  • H.P. HauberEmail author
Schwerpunkt
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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.

Schlüsselwörter

Idiopathische Lungenfibrose Interstitielle Lungenerkrankung Kollagenose Pulmonale Hypertonie Sarkoidose 

Interstitial lung diseases and pulmonary hypertension

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.

Keywords

Connective tissue disease Idopathic pulmonary fibrosis Interstitial lung disease Pulmonary hypertension Sarcoidosis 

Notes

Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin:

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

Literatur

  1. 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: 277–304Google Scholar
  2. 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: 735–740PubMedCrossRefGoogle Scholar
  3. 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–47SPubMedCrossRefGoogle Scholar
  4. 4.
    Behr J, Ryu JH (2008) Pulmonary hypertension in interstitial lung disease. Eur Respir J 31: 1357–1367PubMedCrossRefGoogle Scholar
  5. 5.
    Budhiraja R, Tuder RM, Hassoun PM (2004) Endothelial dysfunction in pulmonary hypertension. Circulation 109: 159–165PubMedCrossRefGoogle Scholar
  6. 6.
    Chang B, Wigley FM, White B, Wise RA (2003) Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 30: 2398–2405PubMedGoogle Scholar
  7. 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: 755–768PubMedCrossRefGoogle Scholar
  8. 8.
    Fartoukh M, Humbert M, Capron F et al. (2000) Sever pulmonary hypertension in histiocytosis X. Am J Respir Crit Care Med 161: 216–223PubMedGoogle Scholar
  9. 9.
    Foley RJ, Metersky ML (2008) Successful treatment of sarcoidosis-associated pulmonary hypertension with bosentan. Respiration 75: 211–214PubMedCrossRefGoogle Scholar
  10. 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: 895–900PubMedCrossRefGoogle Scholar
  11. 11.
    Giad A, Michel RP, Stewart DJ et al. (1993) Expression of endothelin-1 in lungs of patients with cryptogenic fibrosing alveolitis. Lancet 341: 1550–1554CrossRefGoogle Scholar
  12. 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: 650–656PubMedCrossRefGoogle Scholar
  13. 13.
    Handa T, Nagai S, Mikki S et al. (2006) Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 129: 1246–1252PubMedCrossRefGoogle Scholar
  14. 14.
    Hopkins N, McLoughlin P (2002) The structural basis of pulmonary hypertension in chronic lung disease: remodelling, rarefaction or angiogenesis. J Anat 201: 335–348PubMedCrossRefGoogle Scholar
  15. 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: 75–81PubMedCrossRefGoogle Scholar
  16. 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: 219–226Google Scholar
  17. 17.
    Kubo H, Nakayama K, Yanai M et al. (2005) Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 128: 1475–1482PubMedCrossRefGoogle Scholar
  18. 18.
    Lettieri C, Nathan S, Barnett S et al. (2006) Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 129: 746–752PubMedCrossRefGoogle Scholar
  19. 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: 744–750PubMedCrossRefGoogle Scholar
  20. 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: 360–365PubMedCrossRefGoogle Scholar
  21. 21.
    Nadrous HF, Pellikka PA, Krowka MJ et al. (2005) Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 128: 2392–2399CrossRefGoogle Scholar
  22. 22.
    Nunes H, Humbert M, Capron F et al. (2006) Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 61: 68–74PubMedCrossRefGoogle Scholar
  23. 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: 600–607PubMedGoogle Scholar
  24. 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: 600–608PubMedGoogle Scholar
  25. 25.
    Rodman DM, Lindenfeld J (1990) Successful treatment of sarcoidosis-associated pulmonary hypertension with corticosteroids. Chest 97: 500–502PubMedCrossRefGoogle Scholar
  26. 26.
    Ryu JH, Krowka MJ, Pellikka PA et al. (2007) Pulmonary hypertension in patients with interstitial lung diseases. Mayo Clin Proc 82: 342–350PubMedCrossRefGoogle Scholar
  27. 27.
    Sanchez O, Sitbon O, Jais X et al. (2006) Immunosuppressive therapy in connective tissue disease-associated pulmonary arterial hypertension. Chest 130: 182–189PubMedCrossRefGoogle Scholar
  28. 28.
    Sanz J, Kuschnir P, Rius T et al. (2007) Pulmonary arterial hypertension: noninvasive detection with phase-contrast MR imaging. Radiology 243: 70–79PubMedCrossRefGoogle Scholar
  29. 29.
    Strange C, Highland KB (2005) Pulmonary hypertension in interstitial lung disease. Curr Opin Pulm Med 9: 426–430Google Scholar
  30. 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: 880–892PubMedCrossRefGoogle Scholar

Copyright information

© Springer Medizin Verlag 2009

Authors and Affiliations

  1. 1.Medizinische KlinikForschungszentrum BorstelBorstelDeutschland

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