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Lungenfibrosen

Klassifikation, Diagnostik, Therapie

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Zusammenfassung

In den letzten Jahren wurde eine neue Klassifikation der früher unter dem Begriff "idiopathische Lungenfibrose" (IPF) zusammengefassten idiopathischen interstitiellen Pneumonien erarbeitet. Anhand histologischer Merkmale werden verschiedene Subentitäten mit unterschiedlicher prognostischer und klinischer Relevanz eingeteilt. Die Diagnostik verlangt eine Integration klinischer, radiologischer und histopathologischer Befunde. Mit Hilfe des HR-CT kann die Diagnose der häufigsten Subentität, der IPF/UIP, bei passenden klinischen Befunden auch ohne chirurgische Lungenbiopsie ausreichend sicher gestellt werden. Pathogenetisch wird heute das primäre Geschehen bei der IPF in einer Epithelschädigung mit Störung der normalen Wundheilung gesehen. Daraus ergibt sich das therapeutische Dilemma: mittels Immunsuppression ist die Hemmung der primären Fibroblastenaktivität nicht möglich. Da noch keine antifibrotischen Substanzen zur Verfügung stehen, wird als Standardtherapie eine Kombination aus Prednison mit Azathioprin oder Cyclophosphamid empfohlen. Erste antifibrotische Moleküle werden bereits in klinischen Studien geprüft.

Abstract

A new classification of the idiopathic interstitial pneumonia has recently been proposed, defining idiopathic pulmonary fibrosis (IPF) more rigorously and in contrast to further subentities. The new classification is of prognostic and clinical relevance. Diagnosis requires a combined clinical, radiological and pathological effort. In patients with characteristic clinical and HR-CT findings, the diagnosis of IPF can be made with sufficient confidence without surgical lung biopsy. Recent evidence suggests that the primary pathogenetic event in IPF involves epithelial injury and abnormal wound heeling. This explains the ineffectiveness of the usual anti-inflammatory therapy in the majority of patients. Since no antifibrotic drugs are available at present, the recommended standard therapy is a combination of prednisone with azathioprine or cyclophosphamide. New antifibrotic molecules are already tested in clinical trials.

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Authors and Affiliations

  1. Abteilung Pneumologie/Allergologie, Ruhrlandklinik Essen

    U. Costabel

  2. Allgemeine und experimentelle Pathologie, Universität Bochum

    J. Guzman

  3. Abteilung Pneumologie/Allergologie, Ruhrlandklinik, Tüschener Weg 40, 45239, Essen

    U. Costabel

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  1. U. Costabel
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  2. J. Guzman
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Correspondence to U. Costabel.

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Costabel, U., Guzman, J. Lungenfibrosen. Internist 44 (Suppl 1), S35–S43 (2003). https://doi.org/10.1007/s00108-003-0977-6

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  • DOI: https://doi.org/10.1007/s00108-003-0977-6

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