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Atemwegs-zentrierte interstitielle Fibrose

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Seltene Lungenerkrankungen

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Zusammenfassung

Die Atemwegs-zentrierte interstitielle Fibrose ist durch eine peribronchioläre interstitielle Fibrosierung und typische morphologische Veränderungen der kleinen Atemwege gekennzeichnet. Die Ätiologie ist nicht hinreichend geklärt (idiopathisch vs. auslösende inhalative Noxen bzw. Assoziation mit anderen Erkrankungsbildern). Insbesondere die Abgrenzung und das Verhältnis zur exogen allergischen Alveolitis stellen eine Herausforderung dar. Empirisch wird mit systemischen Kortikosteroiden behandelt, randomisierte klinische Therapiestudien fehlen.

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Author information

Authors and Affiliations

  1. Medizinische Klinik V, Klinikum Fulda, Paceliaallee 4, 36043, Fulda, Deutschland

    Prof. Dr. med. Philipp Markart

  2. Justus-Liebig-Universität Gießen, Ludwigstr. 23, 35390, Gießen, Deutschland

    Prof. Dr. med. Malgorzata Wygrecka

Authors
  1. Prof. Dr. med. Philipp Markart
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  2. Prof. Dr. med. Malgorzata Wygrecka
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Corresponding author

Correspondence to Philipp Markart .

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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Markart, P., Wygrecka, M. (2016). Atemwegs-zentrierte interstitielle Fibrose. 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_13

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  • DOI: https://doi.org/10.1007/978-3-662-48419-7_13

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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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