Zusammenfassung
Idiopathische interstitielle Pneumonien (IIPs) werden gemäß der aktuellen ATS/ERS Leitlinie in chronischfibrosierende (IPF, NSIP), 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 NSIP 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 Zigarettenrauch-assoziierten Formen ist die Tabakentwöhnung. Die COP hat im Allgemeinen eine gute Prognose, während die AIP eine hohe Letalität aufweist.
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Kreuter, M., Claussen, M., Costabel, U. (2016). Idiopathische interstitielle Pneumonien jenseits der IPF. In: Kreuter, M., Costabel, U., Herth, F., Kirsten, D. (eds) Seltene Lungenerkrankungen. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48419-7_12
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