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Pulmonale Manifestationen von Vaskulitiden

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Seltene Lungenerkrankungen

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Zusammenfassung

Pulmonale Manifestationen zählen zu den häufigsten Organbeteiligungen der Kleingefäßvaskulitiden, werden aber bei Vaskulitiden kleiner und mittelgroßer Gefäße nur selten beobachtet. Bis zu 80% aller Patienten mit einer Granulomatose mit Polyangiitis (GPA) entwickeln im Krankheitsverlauf eine pulmonale Beteiligung, z. B. in Form pulmonaler Granulome, endobronchialer Stenosen oder einer diffusen alveolären Hämorrhagie (DAH). Eine DAH kann auch bei bis zu 30% aller Patienten mit mikroskopischer Polyangiitis auftreten, bei der granulomatöse Veränderungen per definitionem ausgeschlossen sind. Patienten mit einer eosinophilen Granulomatose mit Polyangiitis (EGPA) erkranken zunächst an einem schweren Asthma bronchiale und entwickeln im weiteren Krankheitsverlauf in bis zu 70% flüchtige pulmonale Infiltrate als Korrelat einer eosinophilen Alveolitis. Aufgrund des regelhaften Multiorganbefalls erfolgen Diagnose und Therapie in enger interdisziplinärer Abstimmung zwischen Pneumologen und Rheumatologen und anderen Fachärzten. Pulmonale Manifestationen erfordern eine potente immunsuppressive Therapie mit Kortikosteroiden in Kombination mit langwirksamen Immunsuppressiva wie Cyclophosphamid oder Rituximab.

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Authors and Affiliations

  1. Klinik für Innere Medizin, Rheumatologie und Immunologie, Klinik Kirchheim, Eugenstrasse 3, 73230, Kirchheim unter Teck, Deutschland

    Prof. Dr. med. Bernhard Hellmich

  2. Medizinische Klinik II (Onkologie, Hämatologie, Klinische Immunologie, Rheumatologie und Pulmologie), Universitätsklinikum Tübingen, Geissweg 3, 72076, Tübingen, Deutschland

    PD Dr. med. Jörg Henes & Dr. med. Jürgen PD. Hetzel

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  1. Prof. Dr. med. Bernhard Hellmich
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Correspondence to Bernhard Hellmich .

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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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Hellmich, B., Henes, J., Hetzel, J.P. (2016). Pulmonale Manifestationen von Vaskulitiden. 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_9

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