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.
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.
Change history
16 January 2019
Erratum zu:
Pneumologe 2018
https://doi.org/10.1007/s10405-018-0205-7
Aufgrund eines Produktionsfehlers wurde in der ursprünglich veröffentlichten Version dieses Beitrags sowohl in der deutschen als auch in der englischen Überschrift der Untertitel „Interstitial pneumonia with autoimmune features …
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E.B. Börner, U. Costabel, T.E. Wessendorf und F. Bonella geben an, dass kein Interessenkonflikt besteht.
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M. Kreuter, Heidelberg
U. Müller-Ladner, Bad Nauheim
T. Wessendorf, Essen
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Boerner, E.B., Costabel, U., Wessendorf, T.E. et al. Differenzialdiagnose interstitieller Lungenerkrankungen. Pneumologe 15, 413–418 (2018). https://doi.org/10.1007/s10405-018-0205-7
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DOI: https://doi.org/10.1007/s10405-018-0205-7
Schlüsselwörter
- Kollagenose
- Idiopathische pulmonale Fibrose
- Autoimmunerkrankung
- Klassifikation
- Diagnostische Techniken und Verfahren