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Aktuelle Klassifikation interstitieller Lungenerkrankungen

Current classification of interstitial pulmonary diseases

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Zusammenfassung

Hintergrund

Die Konsensusklassifikation der idiopathischen interstitiellen Pneumonien (IIP, Lungenfibrose) aus dem Jahr 2002 hat 2013 eine in Teilen grundlegende Revision erfahren.

Fragestellung

Was sind die wesentlichen Kernpunkte des neuen Consensus Statements der American Thoracic Society (ATS)/European Respiratory Society (ERS) von 2013 für die Klassifikation der Lungenfibrosen?

Material und Methode

Auswertung der zur Verfügung stehenden Literatur zum Thema „Lungenfibrose“ in PubMed.

Ergebnisse

Der interdisziplinäre Ansatz (Pneumologie, Radiologie, Pathologie) zur Diagnosefindung wird gestärkt. Die unspezifische interstitielle Pneumonie (NSIP) ist jetzt eine eigenständige, spezifische Lungenfibroseform. Die raucherassoziierte respiratorische Bronchiolitis mit interstitieller Lungenerkrankung (RB-ILD) wird in der Klinik nun häufig ohne Lungenbiopsie auf der Grundlage der Klinik, Computertomographie sowie der bronchoalveolären Lavage diagnostiziert. Der heterogene Verlauf der idiopathischen Lungenfibrose (IPF) wird anerkannt. Der Begriff „akute Exazerbation“ wird nun im Wesentlichen bei akuten Schüben (< 30 Tage) auf dem Boden einer chronischen Fibrose (IPF und NSIP) verwendet. Es wird erkannt, dass es notwendig ist, einen klinischen Algorithmus zur Klassifizierung und für das Management von IIP-Fällen bereitzustellen. Die pleuroparenchymale Fibroelastose (PPFE) wird als seltene eigenständige spezifische Lungenfibroseform anerkannt. Ein sich schnell entwickelndes Gebiet der molekularen Marker ist vielversprechend für eine Verbesserung der diagnostischen Ansätze.

Schlussfolgerungen

Das ATS-/ERS-Update ist eine Ergänzung zum vorangegangenen Klassifizierung der Lungenfibrosen von 2002. Es umreißt Fortschritte in den letzten Jahren und zeigt potenzielle Bereiche für künftige Neuerungen auf.

Abstract

Background

The consensus classification of idiopathic interstitial pneumonia (IIP, also known as pulmonary fibrosis) in 2002 has undergone a significant revision in 2013.

Objectives

What are the key points of the new consensus statement of the American Thoracic Society (ATS)/European Respiratory Society (ERS) from 2013 for the classification of pulmonary fibrosis?

Material and methods

Evaluation of the available literature on the subject of pulmonary fibrosis in Pubmed.

Results

The interdisciplinary approach (i.e. pulmonology, radiology and pathology) for the diagnosis of lung fibrosis subtypes is highlighted. Nonspecific interstitial pneumonia (NSIP) is now a separate specific form of pulmonary fibrosis. Smoking-associated respiratory bronchiolitis with interstitial lung disease (RB-ILD) is now frequently diagnosed without a lung biopsy on the basis of clinical findings, computed tomography and bronchoalveolar lavage. The heterogeneous course of idiopathic pulmonary fibrosis (IPF) is now recognized. The term “acute exacerbation” will now be used for acute attacks (< 30 days) in patients with chronic lung fibrosis (IPF and NSIP). It has been recognized that it is necessary to provide a clinical classification algorithm for the management of IIP cases. Pleuroparenchymal fibroelastosis (PPFE) is recognized as a rare new entity of pulmonary fibrosis. The rapidly evolving field of molecular markers is promising for improving the diagnostic approach.

Conclusion

The 2013 ATS/ERS update is a supplement to the previous classification of lung fibrosis from 2002. It outlines progress in recent years and shows potential areas for future innovations.

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Literatur

  1. Ryerson CJ, Collard HR (2013) Update on the diagnosis and classification of ILD. Curr Opin Pulm Med 19:453–459

    Article  PubMed  Google Scholar 

  2. Bradley B, Branley HM, Egan JJ et al (2008) Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 63(Suppl 5):v1–v58

    Article  PubMed  Google Scholar 

  3. Baughman RP, Culver DA, Judson MA (2011) A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med 183:573–581

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  4. Travis WD, Costabel U, Hansell DM et al (2013) An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 188:733–748

    Article  PubMed  Google Scholar 

  5. American Thoracic Society, European Respiratory Society (2002) American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ats board of directors, june 2001 and by the ERS executive committee, june 2001. Am J Respir Crit Care Med 165:277–304

    Article  Google Scholar 

  6. 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 

  7. Sumikawa H, Johkoh T, Colby TV et al (2008) Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med 177:433–439

    Article  PubMed  Google Scholar 

  8. Travis WD, Hunninghake G, King TE Jr et al (2008) Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med 177:1338–1347

    Article  PubMed  Google Scholar 

  9. Akira M, Inoue Y, Arai T et al (2011) Long-term follow-up high-resolution ct findings in non-specific interstitial pneumonia. Thorax 66:61–65

    Article  PubMed  Google Scholar 

  10. Park IN, Jegal Y, Kim DS et al (2009) Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J 33:68–76

    Article  CAS  PubMed  Google Scholar 

  11. Hidalgo A, Franquet T, Gimenez A et al (2006) Smoking-related interstitial lung diseases: radiologic-pathologic correlation. Eur Radiol 16:2463–2470

    Article  PubMed  Google Scholar 

  12. Craig PJ, Wells AU, Doffman S et al (2004) Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking. Histopathology 45:275–282

    Article  CAS  PubMed  Google Scholar 

  13. Fraig M, Shreesha U, Savici D, Katzenstein AL (2002) Respiratory bronchiolitis: a clinicopathologic study in current smokers, ex-smokers, and never-smokers. Am J Surg Pathol 26:647–653

    Article  PubMed  Google Scholar 

  14. Vassallo R, Ryu JH (2008) Tobacco smoke-related diffuse lung diseases. Semin Respir Crit Care Med 29:643–650

    Article  PubMed  Google Scholar 

  15. Portnoy J, Veraldi KL, Schwarz MI et al (2007) Respiratory bronchiolitis-interstitial lung disease: long-term outcome. Chest 131:664–671

    Article  PubMed  Google Scholar 

  16. Bullard JE, Wert SE, Whitsett JA et al (2005) ABCA3 mutations associated with pediatric interstitial lung disease. Am J Respir Crit Care Med 172:1026–1031

    Article  PubMed Central  PubMed  Google Scholar 

  17. Oymak FS, Demirbas HM, Mavili E et al (2005) Bronchiolitis obliterans organizing pneumonia. Clinical and roentgenological features in 26 cases. Respiration 72:254–262

    Article  PubMed  Google Scholar 

  18. Lee JW, Lee KS, Lee HY et al (2010) Cryptogenic organizing pneumonia: serial high-resolution ct findings in 22 patients. AJR Am J Roentgenol 195:916–922

    Article  PubMed  Google Scholar 

  19. Lazor R, Vandevenne A, Pelletier A et al (2000) Cryptogenic organizing pneumonia. Characteristics of relapses in a series of 48 patients. The groupe d’etudes et de recherche sur les maladles „orphelines“ pulmonaires (germ“o“p). Am J Respir Crit Care Med 162:571–577

    Article  CAS  PubMed  Google Scholar 

  20. Ichikado K, Suga M, Muller NL et al (2002) Acute interstitial pneumonia: comparison of high-resolution computed tomography findings between survivors and nonsurvivors. Am J Respir Crit Care Med 165:1551–1556

    Article  PubMed  Google Scholar 

  21. Collard HR, Moore BB, Flaherty KR et al (2007) Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 176:636–643

    Article  PubMed Central  PubMed  Google Scholar 

  22. Beasley MB, Franks TJ, Galvin JR et al (2002) Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage. Arch Pathol Lab Med 126:1064–1070

    PubMed  Google Scholar 

  23. Churg A, Myers J, Suarez T et al (2004) Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol 28:62–68

    Article  PubMed  Google Scholar 

  24. Cha SI, Fessler MB, Cool CD et al (2006) Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur Respir J 28:364–369

    Article  PubMed  Google Scholar 

  25. Reddy TL, Tominaga M, Hansell DM et al (2012) Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 40:377–385

    Article  PubMed  Google Scholar 

  26. Vassallo R, Jensen EA, Colby TV et al (2003) The overlap between respiratory bronchiolitis and desquamative interstitial pneumonia in pulmonary langerhans cell histiocytosis: high-resolution CT, histologic, and functional correlations. Chest 124:1199–1205

    Article  PubMed  Google Scholar 

  27. 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 

  28. Ryerson CJ, Urbania TH, Richeldi L et al (2013) Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J 42:750–757

    Article  PubMed  Google Scholar 

  29. Prasse A, Probst C, Bargagli E et al (2009) Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 179:717–723

    Article  CAS  PubMed  Google Scholar 

  30. Prasse A, Pechkovsky DV, Toews GB et al (2007) CCl18 as an indicator of pulmonary fibrotic activity in idiopathic interstitial pneumonias and systemic sclerosis. Arthritis Rheum 56:1685–1693

    Article  CAS  PubMed  Google Scholar 

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Einhaltung ethischer Richtlinien

Interessenkonflikt. J. Vogel-Claussen und A. Prasse geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Vogel-Claussen, J., Prasse, A. Aktuelle Klassifikation interstitieller Lungenerkrankungen. Radiologe 54, 1147–1152 (2014). https://doi.org/10.1007/s00117-014-2732-5

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  • DOI: https://doi.org/10.1007/s00117-014-2732-5

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