Akute Exazerbation der idiopathischen Lungenfibrose

Die idiopathische Lungenfibrose (IPF) zählt zu den am häufigsten vorkommenden fibrosierenden interstitiellen Lungenerkrankungen. Eine relevante Komplikation der IPF stellt die akute Exazerbation (AE-IPF) dar, die mit einer hohen Letalität assoziiert ist. Dieser Beitrag soll Informationen zu dem Krankheitsbild einer AE-IPF liefern und die heute zur Verfügung stehenden diagnostischen, therapeutischen und präventiven Maßnahmen erläutern.

Abstract

Acute exacerbations (AE) are a life-threatening complication in patients with idiopathic pulmonary fibrosis (IPF). In-hospital mortality is high and the overall prognosis poor. The underlying causes of AE-IPF still remain unclear and there are no focused guidelines for its management. In most cases high-dose steroids combined with an antibiotic therapy are applied. Preventive and palliative measures are very important. Intensive research is necessary to improve management of AE-IPF.

Kernaussagen

• Die AE-IPF gilt als eine lebensgefährliche Komplikation der IPF, da sie mit einer hohen intrahospitalen Letalität und schlechten Prognose einhergeht.

• Die Ursachen sind noch unzureichend verstanden, und eindeutige therapeutische Empfehlungen gibt es bislang nicht.

• Die Hochdosiskortisontherapie, kombiniert mit einer antibiotischen Therapie, wird in den meisten Fällen eingesetzt.

• Evidenzgesicherte Therapieformen existieren bisher nicht.

• Zu den wichtigsten präventiven Maßnahmen gehören Impfungen und Antifibrotika.

• Palliative Maßnahmen, insbesondere die Linderung von Dyspnoe und Husten, sind im Anschluss an eine AE-IPF frühzeitig bei Bedarf einzuleiten.

Schlüsselwörter

akute Exazerbation - idiopathische Lungenfibrose - interstitielle Lungenerkrankung

Keywords

acute exacerbation - idiopathic pulmonary fibrosis - interstitial lung disease

Publication History

Article published online:
09 August 2021

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