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Grundlagen, Epidemiologie und Pathogenese der idiopathischen Lungenfibrose

Basic principles, epidemiology and pathogenesis of idiopathic pulmonary fibrosis

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Zusammenfassung

Die idiopathische Lungenfibrose (IPF) ist die häufigste Form der Lungenfibrosen (interstitielle Pneumonien). Sie geht mit einer schlechten Prognose und einem mittleren Überleben von 28 Monaten einher. Es werden eine familiäre und eine sporadische Form unterschieden. Die Pathogenese der sporadischen Form wird durch zahlreiche Faktoren beeinflusst. Den familiären Formen liegt häufig eine Genmutation zugrunde. Als Risikofaktoren für die sporadische Form gelten vorangeschrittenes Alter, männliches Geschlecht, Zigarettenrauchen, Staubexposition und Mikroaspiration. Pathogenetisch liegt eine fehlgeleitete Wundheilung mit aberranten Reparaturprozessen der IPF zugrunde.

Abstract

Within the group of idiopathic interstitial pneumonitis, idiopathic pulmonary fibrosis (IPF) is the most frequent form and is a devastating disease with a mean survival time of 28 months. Familiar and sporadic variants are described. While familiar IPF is often caused by gene mutations, evolution of sporadic IPF is thought to be influenced by multiple factors including genetics. Risk factors for sporadic IPF are advanced age, male gender, cigarette smoking, dust exposure and microaspiration. The underlying pathomechanisms of IPF are derailed wound healing and aberrant repair processes.

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  1. Klinik für Pneumologie, Universitätsklinikum Freiburg, Killianstr. 5, 79106, Freiburg, Deutschland

    A. Prasse & J. Müller-Quernheim

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  1. A. Prasse
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  2. J. Müller-Quernheim
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Correspondence to J. Müller-Quernheim.

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Prasse, A., Müller-Quernheim, J. Grundlagen, Epidemiologie und Pathogenese der idiopathischen Lungenfibrose. Pneumologe 10, 81–88 (2013). https://doi.org/10.1007/s10405-012-0605-z

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