Interstitielle Lungenerkrankungen (ILD) führen oftmals zu einer progredienten Fibrosierung der Lunge und damit zu entsprechenden Funktionseinschränkungen. Bei der rasch fortschreitenden idiopathischen pulmonalen Fibrose (IPF) sind antifibrotische Medikamente bereits seit einigen Jahren in der Therapie etabliert. Der Beitrag bietet eine Übersicht über den Einsatz antifibrotischer Medikamente, sowohl bei der IPF als auch bei den speziellen non-IPF-ILDs.
Abstract
The interstitial lung diseases ILDs are a heterogeneous group of diseases that often lead to progressive fibrosis of the lungs with corresponding functional impairment. With nintedanib, a tyrosinkinase inhibitor and angiokinase inhibitor, as well as pirfenidone, which unfolds its effect among other things by inhibiting the transforming growth factor β, there are currently 2 approved antifibrotic drugs. In the rapidly progressing idiopathic pulmonary fibrosis IPF, the antifibrotic drugs nintedanib and pirfenidone have been established and approved in therapy for several years. The initiation of antifibrotic therapy should be carried out early after diagnosis by multidisciplinary discussion (MDD). In systemic scleroderma with lung involvement nintedanib should be used in the case of relevant fibrosis in addition to immunosuppressive therapy. Recently, nintedanib has also become a new option for the treatment of progressive fibrosing ILDs (PF-ILDs). This describes the course of various disease entities such as connective tissue disease associated ILDs (CTD-ILDs), fibrosing hypersensitivity pneumonitis or fibrosing courses of non-IPF idiopathic interstitial pneumonitis (non-IPF IIPs) that have a corresponding fibrose-related worsening of respiratory symptoms, a deterioration of lung-functioning parameters or a disease progression in CT. Although pirfenidone also shows positive signals for this group of patients in some selected studies, its use in PF-ILD is not yet recommended. In particular, gastrointestinal side effects can occur under therapy with antifibrotic drugs and require a long-term close interdisciplinary connection of patients.
Schlüsselwörter
interstitielle Lungenerkrankungen - antifibrotische Therapie - Nintedanib, Pirfenidon
Abstract
interstitial lung disease - antifibrotic therapy - nintedanib - pirfenidone
