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Pneumologie 2015; 69(10): 608-615
DOI: 10.1055/s-0034-1393036
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© Georg Thieme Verlag KG Stuttgart · New York

Die idiopathische Lungenfibrose

Idiopathic Pulmonary Fibrosis
A. Prasse
1   Medizinische Hochschule Hannover, Abteilung für Pneumologie
2   Fraunhofer ITEM; Clinical Research Center Hannover
› Author Affiliations
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Publication History

Publication Date:
07 October 2015 (online)

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Zusammenfassung

Die idiopathische Lungenfibrose (IPF) ist die häufigste Form der Lungenfibrosen und tritt v. a. im höheren Lebensalter auf. Neben Alterungsprozessen und der genetischen Veranlagung ist ein wichtiger Risikofaktor das Zigarettenrauchen und die Exposition gegenüber lungentoxischen Substanzen. Der IPF liegt das Muster der Usual Interstitial Pneumonitis (UIP) histologisch zugrunde. Die Diagnosestellung ist komplex und sollte multidisziplinär im Ausschluss anderweitiger Erkrankungen erfolgen. Der einzige kurative Ansatz ist die Lungentransplantation. In den letzten Jahren hat es in der medikamentösen Therapie einen Durchbruch gegeben, erstmals wurden zwei Medikamente, Pirfenidon und Nintedanib, für die Behandlung der IPF zugelassen.

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF.

 

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