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2022 | OriginalPaper | Buchkapitel

12. Idiopathische interstitielle Pneumonien jenseits der IPF

verfasst von : Michael Kreuter, Martin Claussen, Ulrich Costabel

Erschienen in: Seltene Lungenerkrankungen

Verlag: Springer Berlin Heidelberg

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Zusammenfassung

Idiopathische interstitielle Pneumonien (IIP) werden gemäß der aktuellen ATS/ERS-Leitlinie in chronisch fibrosierende (IPF, NSIP), mit Zigarettenrauch assoziierte (RB-ILD, DIP), akute/subakute (AIP, COP) sowie seltene (LIP, PPFE) und unklassifizierbare Formen unterteilt. Essenziell ist bei allen Entitäten der Ausschluss bekannter Ursachen und Assoziationen (Kollagenosen, Medikamente, exogen-allergische Alveolitis). Nach der IPF (idiopathische Lungenfibrose) ist die idiopathische NSIP (nichtspezifische interstitielle Pneumonie)die zweithäufigste IIP. Diese wird in einen zellulären und fibrotischen Subtyp unterteilt und spricht je nach Typ relativ gut auf Immunsuppression an. Wesentliche Therapie der zigarettenrauchassoziierten Formen ist die Tabakentwöhnung. Die kryptogen organisierende Pneumonie (COP) hat im Allgemeinen eine gute Prognose, während die AIP eine sehr hohe Letalität aufweist.

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Titel: Idiopathische interstitielle Pneumonien jenseits der IPF
verfasst von: Michael Kreuter
Martin Claussen
Ulrich Costabel
Verlag: Springer Berlin Heidelberg
Buch: Seltene Lungenerkrankungen
Print ISBN: 978-3-662-63650-3

Electronic ISBN: 978-3-662-63651-0

Copyright-Jahr: 2022
DOI: https://doi.org/10.1007/978-3-662-63651-0_12

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