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DOI: 10.1055/a-1273-4105
Akute Exazerbation der idiopathischen Lungenfibrose
Exacerbation in Idiopathic Pulmonary FibrosisDie 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.
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Die AE-IPF gilt als eine lebensgefährliche Komplikation der IPF, da sie mit einer hohen intrahospitalen Letalität und schlechten Prognose einhergeht.
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Die Ursachen sind noch unzureichend verstanden, und eindeutige therapeutische Empfehlungen gibt es bislang nicht.
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Die Hochdosiskortisontherapie, kombiniert mit einer antibiotischen Therapie, wird in den meisten Fällen eingesetzt.
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Evidenzgesicherte Therapieformen existieren bisher nicht.
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Zu den wichtigsten präventiven Maßnahmen gehören Impfungen und Antifibrotika.
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Palliative Maßnahmen, insbesondere die Linderung von Dyspnoe und Husten, sind im Anschluss an eine AE-IPF frühzeitig bei Bedarf einzuleiten.
Publication History
Article published online:
09 August 2021
© 2021. Thieme. All rights reserved.
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