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Die Therapie der idiopathischen pulmonalen Fibrose

Besonderheiten und Komplikationen

Therapy of idiopathic pulmonary fibrosis

Special features and complications

  • Leitthema
  • Published:
Der Pneumologe Aims and scope

Zusammenfassung

Die Beratung und die Therapie von Patienten mit idiopathischer pulmonaler Fibrose (IPF) konfrontieren den Arzt mit einigen krankheitstypischen Problemen. Der folgende Artikel geht ein auf die Besonderheiten hinsichtlich des Krankheitsverlaufs, inklusive der „akuten Exazerbation“, möglicher Komplikationen wie z. B. Lungenembolie oder respiratorische Infekte, Komorbiditäten wie z. B. interstitielle Lungenerkrankung und pulmonale Hypertonie (ILD-PH), gastroösophagealer Reflux (GER) sowie resultierender Therapieentscheidungen, beispielweise einer etwaigen vasoaktiven Therapie oder einer Refluxbehandlung, aber auch einer supportiven Therapie (O2-Gabe) oder palliativer Maßnahmen und orientiert sich dabei u. a. an der neu erstellten internationalen Leitlinie und ihrer aktuellen deutschen Adaption.

Abstract

This article focuses on specific characteristics that are encountered by physicians when treating patients with idiopathic pulmonary fibrosis (IPF). This characterized by its highly variable clinical course which is strongly influenced by clinical deterioration termed acute exacerbations and certain comorbid conditions, such as pulmonary embolism, gastroesophageal reflux (GER), respiratory infections or secondary interstitial lung disease and pulmonary arterial hypertension (ILD-PH). Treatment decisions comprise among others the initiation or discontinuation of specific vasoactive therapy for ILD-PH, the treatment of GER and supportive care (long-term oxygen therapy) or palliative treatment.

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Literatur

  1. Carrington CB, Gaensler EA, Coutu RE et al (1978) Natural history and treated course of usual and desquamative interstitial pneumonia. N Engl J Med 298:801–809

    Article  PubMed  CAS  Google Scholar 

  2. Ley B, Collard HR, King TE Jr (2011) Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 183:431–440

    Article  PubMed  Google Scholar 

  3. Bois RM du, Weycker D, Albera C (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 

  4. Swigris JJ, Wamboldt FS, Behr J et al (2010) The 6 minute walk in idiopathic pulmonary fibrosis: longitudinal changes and minimum important difference. Thorax 65:173–177

    Article  PubMed  Google Scholar 

  5. Kim DS, Park JH, Park BK et al (2006) Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J 27:143–150

    Article  PubMed  CAS  Google Scholar 

  6. Parambil JG, Myers JL, Ryu JH (2005) Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest 128:3310–3315

    Article  PubMed  Google Scholar 

  7. Collard HR, Moore BB, Flaherty KR et al (2007) Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 176:636–643

    Article  PubMed  Google Scholar 

  8. Panos RJ, Mortenson RL, Niccoli SA et al (1990) Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med 88:396–404

    Article  PubMed  CAS  Google Scholar 

  9. Wootton SC, Kim DS, Kondoh Y et al (2011) Viral infection in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 183:1698–1702

    Article  PubMed  Google Scholar 

  10. Yuksel M, Ozyurtkan MO, Bostanci K et al (2006) Acute exacerbation of interstitial fibrosis after pulmonary resection. Ann Thorac Surg 82:336–338

    Article  PubMed  Google Scholar 

  11. Hiwatari N, Shimura S, Takishima T et al (1994) Bronchoalveolar lavage as a possible cause of acute exacerbation in idiopathic pulmonary fibrosis patients. Tohoku J Exp Med 174:379–386

    Article  PubMed  CAS  Google Scholar 

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

    Google Scholar 

  13. Umeda Y, Morikawa M, Anzai M (2010) Acute exacerbation of idiopathic pulmonary fibrosis after pandemic influenza A (H1N1) vaccination. Intern Med 49:2333–2336

    Article  PubMed  Google Scholar 

  14. Raghu G, Collard HR, Egan JJ et al (2011) 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 

  15. Behr J, Günther A, Ammenwerth W et al (2013) S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose. Pneumologie [Epub ahead of print]

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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  18. Hoeper MM, Ghofrani HA, Gorenflo M et al (2010) Diagnostik und Therapie der pulmonalen Hypertonie: Europäische Leitlinie 2009. Pneumologie 64:401–414

    Article  PubMed  CAS  Google Scholar 

  19. Ghofrani HA, Distler O, Gerhardt F et al (2010) Treatment of pulmonary arterial hypertension (PAH): recommendations of the Cologne Consensus Conference 2010. Dtsch Med Wochenschr 135(Suppl 3):87–101

    Article  Google Scholar 

  20. 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 

  21. Minai OA, Sahoo D, Chapman JT et al (2008) Vaso-active therapy can improve 6-min walk distance in patients with pulmonary hypertension and fibrotic interstitial lung disease. Respir Med 102:1015–1020

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  23. Madden BP, Allenby M, Loke T et al (2006) A potential role for sildenafil in the management of pulmonary hypertension in patients with parenchymal lung disease. Vascul Pharmacol 44:372–376

    Article  PubMed  CAS  Google Scholar 

  24. Collard HR, Anstrom KJ, Schwarz MI et al (2007) Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest 131:897–899

    Article  PubMed  Google Scholar 

  25. Hoeper MM, Halank M, Wilkens H et al (2012) Riociguat for interstitial lung disease and pulmonary hypertension: a pilot trial. Eur Respir J (in press) DOI 10.1183/09031936.00213911

  26. 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 

  27. Morrison DA, Stovall JR (1992) Increased exercise capacity in hypoxemic patients after long-term oxygen therapy. Chest 102:542–550

    Article  PubMed  CAS  Google Scholar 

  28. Nocturnal Oxygen Therapy Trial Group (1980) Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Ann Intern Med 93:391–398

    Google Scholar 

  29. o A (1981) Longterm domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema: report of the Medical Research Council Working Party. Lancet 1:681–686

    Google Scholar 

  30. Stern JB, Mal H, Groussard O et al (2001) Prognosis of patients with advanced idiopathic pulmonary fibrosis requiring mechanical ventilation for acute respiratory failure. Chest 120:213–219

    Article  PubMed  CAS  Google Scholar 

  31. Mollica C, Paone G, Conti V et al (2010) Mechanical ventilation in patients with end-stage idiopathic pulmonary fibrosis. Respiration 79:209–215

    Article  PubMed  Google Scholar 

  32. Mason DP, Thuita L, Nowicki ER et al (2010) Should lung transplantation be performed for patients on mechanical respiratory support? The US experience. J Thorac Cardiovasc Surg 139:765–773

    Article  PubMed  Google Scholar 

  33. Vogelmeier C, Buhl R, Criée CP et al (2007) Guidelines for the diagnosis and therapy of COPD issued by Deutsche Atemwegsliga and Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin. Pneumologie 61:e1–e40

    Article  PubMed  CAS  Google Scholar 

  34. Vestbo J, Hurd SS, Agusti AG et al (2012) Global Strategy for the Diagnosis, Management and Prevention of Chronic Obstructive Pulmonary Disease, GOLD Executive Summary. Am J Respir Crit Care Med (in press) DOI 10.1164/rccm.201204-0596PP

  35. Nishiyama O, Kondoh Y, Kimura T (2008) Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology 13:394–399

    Article  PubMed  Google Scholar 

  36. Ferreira A, Garvey C, Connors GL (2009) Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response. Chest 135:442–447

    Article  PubMed  Google Scholar 

  37. Ferreira G, Feuerman M, Spiegler P (2006) Results of an 8-week, outpatient pulmonary rehabilitation program on patients with and without chronic obstructive pulmonary disease. J Cardiopulm Rehabil 26:54–60

    Article  PubMed  Google Scholar 

  38. Raghu G, Freudenberger TD, Yang S et al (2006) High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J 27:136–142

    Article  PubMed  CAS  Google Scholar 

  39. Tobin RW, Pope CE II, Pellegrini CA et al (1998) Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 158:1804–1808

    PubMed  CAS  Google Scholar 

  40. Marik PE (2001) Aspiration pneumonitis and aspiration pneumonia. N Engl J Med 344:665–671

    Article  PubMed  CAS  Google Scholar 

  41. Barnes TW, Vassallo R, Tazelaar HD et al (2006) Diffuse bronchiolar disease due to chronic occult aspiration. Mayo Clin Proc 81:172–176

    Article  PubMed  Google Scholar 

  42. Linden PA, Gilbert RJ, Yeap BY et al (2006) Laparoscopic fundoplication in patients with end-stage lung disease awaiting transplantation. J Thorac Cardiovasc Surg 131:438–446

    Article  PubMed  Google Scholar 

  43. Raghu G, Freudenberger TD, Yang S et al (2006) High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J 27:136–142

    Article  PubMed  CAS  Google Scholar 

  44. Mejia M, Carrillo G, Rojas-Serrano J et al (2009) Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 136:10–15

    Article  PubMed  Google Scholar 

  45. Cottin V, Nunes H, Brillet PY et al (2005) Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 26:586–593

    Article  PubMed  CAS  Google Scholar 

  46. Lancaster LH, Mason WR, Parnell JA et al (2009) Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest 136:772–778

    Article  PubMed  Google Scholar 

  47. Allen S, Raut S, Woollard J et al (2005) Low dose diamorphine reduces breathlessness without causing a fall in oxygen saturation in elderly patients with end-stage idiopathic pulmonary fibrosis. Palliat Med 19:128–130

    Article  PubMed  Google Scholar 

  48. Lee JS, McLaughlin S, Collard HR (2011) Comprehensive care of the patient with idiopathic pulmonary fibrosis. Curr Opin Pulm Med 17:348–354

    Article  PubMed  Google Scholar 

  49. Hope-Gill BDM, Hilldrup S, Davies C et al (2003) A study of the cough reflex in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 168:995–1002

    Article  PubMed  Google Scholar 

  50. Horton MR, Danoff SK, Lechtzin N (2008) Thalidomide inhibits the intractable cough of idiopathic pulmonary fibrosis. Thorax 63:749

    Article  PubMed  CAS  Google Scholar 

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: Prof. A. Günther: Actelion, Activero, Boehringer Ingelheim, InterMune, Novartis, Nycomed, Pfizer; Prof. P. Markart: Boehringer Ingelheim, InterMune.

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Authors and Affiliations

  1. Standort Giessen, Medizinische Klinik II, Pneumologie und Intensivmedizin, Universitätsklinikum Gießen Marburg GmbH, Klinikstr. 33, 35392, Gießen, Deutschland

    D. von der Beck, P. Markart, W. Seeger &  A. Günther

  2. Pneumologische Klinik Waldhof-Elgershausen, Greifenstein, Deutschland

    A. Günther

  3. Mitglied im Deutschen Zentrum für Lungenforschung (DZL), München, Deutschland

    W. Seeger &  A. Günther

Authors
  1. D. von der Beck
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  2. P. Markart
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  3. W. Seeger
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  4. A. Günther
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Correspondence to A. Günther.

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von der Beck, D., Markart, P., Seeger, W. et al. Die Therapie der idiopathischen pulmonalen Fibrose. Pneumologe 10, 105–111 (2013). https://doi.org/10.1007/s10405-012-0608-9

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