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Pulmonale Langerhans-Zell-Histiozytose

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Seltene Lungenerkrankungen

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Zusammenfassung

Histomorphologisches Korrelat der pulmonalen Langerhans-Zell-Granulomatose sind nekrotisierende Granulome. Im CT zeigt sich charakteristischerweise das Muster dickwandiger, wie zufällig verteilter Zysten im Lungenparenchym. Oft klingt die Erkrankung spontan ab und heilt narbig aus. Bei stärkerem Befall kann sich eine einer COPD ähnliche Einschränkung der Lungenfunktion mit permanenter Atemwegsobstruktion und Überblähung entwickeln. Wenn eine Therapie in der aktiven entzündlichen Phase notwendig erscheint, sind systemische Kortikosteroide beim Erwachsenen zuverlässig wirksam. Ein Rauchstopp sollte für jeden Patienten obligat sein, da die Krankheit fast ausschließlich Raucher betrifft.

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Author information

Authors and Affiliations

  1. Pneumologie - Lungenklinik Heckeshorn, Helios Klinikum Emil von Behring, Walterhöferstr. 11, 14165, Berlin, Deutschland

    Dr. med. Nicolas Schönfeld

  2. vormals LungenClinic, Großhansdorf, Deutschland

    Prof. Dr. med. Detlef Kirsten

Authors
  1. Dr. med. Nicolas Schönfeld
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  2. Prof. Dr. med. Detlef Kirsten
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Corresponding author

Correspondence to Nicolas Schönfeld .

Editor information

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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Schönfeld, N., Kirsten, D. (2016). Pulmonale Langerhans-Zell-Histiozytose. 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_16

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  • DOI: https://doi.org/10.1007/978-3-662-48419-7_16

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-48418-0

  • Online ISBN: 978-3-662-48419-7

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