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Der Pneumologe

, Volume 15, Issue 6, pp 413–418 | Cite as

Differenzialdiagnose interstitieller Lungenerkrankungen

  • E. B. BoernerEmail author
  • U. Costabel
  • T. E. Wessendorf
  • F. Bonella
Leitthema
  • 494 Downloads

Zusammenfassung

In den letzten Jahren haben sich die Klassifikationen der interstitiellen Lungenerkrankungen (ILD) und die Therapie der idiopathischen Lungenfibrose (IPF) grundlegend gewandelt. Da sich die Therapie der IPF von der anderer ILDs abgrenzt, ist es umso wichtiger, andere fibrosierende ILDs richtig zu diagnostizieren. Die Bezeichnung „Interstitial pneumonia with autoimmune features“ (IPAF) und deren Klassifikationskriterien wurden 2015 von einer ERS/ATS-Task-Force im Rahmen einer Konsensusfindung vorgeschlagen, um Patienten mit interstitiellen Pneumonien und Eigenschaften einer Autoimmunerkrankung zu beschreiben, die nicht die definitiven Kriterien einer Kollagenose erfüllen. Die Therapie ist aktuell weiterhin in erster Linie immunsuppressiv; prospektive Daten hierzu fehlen jedoch.

Schlüsselwörter

Kollagenose Idiopathische pulmonale Fibrose Autoimmunerkrankung Klassifikation Diagnostische Techniken und Verfahren 

Differential diagnosis of interstitial lung disease

Abstract

In recent years, substantial progress has been made regarding the classification of idiopathic interstitial pneumonia (IIP) and the treatment of idiopathic pulmonary fibrosis (IPF). As the treatment of IPF is different from that of other IIPs, it has become more important to correctly diagnose other fibrotic IIPs. In 2015, an ERS/ATS task force proposed the term “interstitial pneumonia with autoimmune features” (IPAF) in order to create a consensus regarding the nomenclature and classification criteria for an interstitial pneumonia suggestive of an underlying autoimmune cause without, however, fulfilling the definitive criteria for a connective tissue disease (CTD). The treatment options are currently immunosuppressive agents, but prospective data are still missing.

Keywords

Connective tissue disease Idiopathic pulmonary fibrosis Autoimmune disease Classification Diagnostic techniques and procedures 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

E.B. Börner, U. Costabel, T.E. Wessendorf und F. Bonella geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  • E. B. Boerner
    • 1
    Email author
  • U. Costabel
    • 1
  • T. E. Wessendorf
    • 1
  • F. Bonella
    • 1
  1. 1.Klinik für Pneumologie, Schwerpunkt interstitielle und seltene LungenkrankheitenUniversitätsmedizin Essen – RuhrlandklinikEssenDeutschland

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