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Idiopathische Lungenfibrose

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Book cover Seltene Lungenerkrankungen

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Zusammenfassung

Die idiopathische Lungenfibrose ist eine chronisch progrediente fibrosierende interstitielle Pneumonie unbekannter Ursache, die primär beim älteren Erwachsenen auftritt und sich radiologisch sowie histologisch durch das Vorliegen eines UIP-Musters (usual interstitial pneumonia) auszeichnet. Die Prognose ist ungünstig und mit der verschiedener onkologischer Erkrankungen vergleichbar. Interindividuell ist der Verlauf jedoch sehr variabel. Die zentrale Säule der Diagnostik ist die hochauflösende Computertomografie, eine Histologiegewinnung ist nur selten erforderlich. Die Diagnosestellung sollte in einer interdisziplinären Fallevaluation erfolgen. Therapeutisch stellt die Lungentransplantation die einzige kurative Option dar. Mit den antifibrotisch wirksamen Substanzen Pirfenidon oder Nintedanib kann eine Progressionsverzögerung erreicht werden. Supportive Ansätze sollten früh in die Behandlung integriert werden.

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Authors and Affiliations

  1. Pneumologie, Krankenhaus Bethanien, Aufderhöher Str. 169–175, 42699, Solingen, Deutschland

    Dr. med. Lars Hagmeyer

  2. Firestone Institute for Respiratory Health, McMaster University Hamilton, 50 Charlton Avenue East, T2131, L8N 4A6, Hamilton, Ontario, Kanada

    Prof. Dr. med. Martin Kolb

Authors
  1. Dr. med. Lars Hagmeyer
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  2. Prof. Dr. med. Martin Kolb
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Correspondence to Lars Hagmeyer .

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Editors and Affiliations

  1. Pneumologie und Beatmungsmediz, Universitätsklinikum Heidelberg, Heidelberg, Baden-Württemberg, Germany

    Michael Kreuter

  2. Ruhrlandklinik, Universitätsklinikum Essen, Essen, Nordrhein-Westfalen, Germany

    Ulrich Costabel

  3. Universitätsklinikum Heidelberg, Heidelberg, Baden-Württemberg, Germany

    Felix Herth

  4. Zentrum für Pneumologie & Thoraxchir., LungenClinic Großhansdorf, Großhansdorf, Schleswig-Holstein, Germany

    Detlef Kirsten

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Hagmeyer, L., Kolb, M. (2016). Idiopathische Lungenfibrose. 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_11

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