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LAM: Neue Therapien – neue Chancen?

LAM: new therapies – new chances?

  • Leitthema
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Zusammenfassung

Die Lymphangioleiomyomatose (LAM) ist eine seltene Lungenkrankheit und befällt fast ausschließlich Frauen. Die LAM wird heute zu den proliferativen Krankheiten gerechnet. In den vergangen 15 Jahren wurden sehr große Fortschritte im Verständnis der komplexen zellulären Pathomechanismen gemacht. Daraus hat sich seit einigen Jahren eine wichtige Therapie entwickelt, mit der es gelingt, die Krankheit bei vielen Patientinnen aufzuhalten. Des Weiteren gibt es zusätzliche Therapieansätze, die auf die mTOR-Inhibition aufbauen und darauf abzielen, die mutierten TSC2−/−-Zellen weiter zu schädigen bzw. Prozesse, wie eine Steigerung der Autophagie oder Verminderung der Apoptose, die nach Gabe des mTOR-Inhibitors auch eintreten, zu verhindern. Diese Ansätze lassen eine Heilung in Zukunft als möglich erscheinen. Der Beitrag fasst die wichtigsten Therapieansätze auf diesem Gebiet zusammen.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare lung disease that is almost exclusively seen in women and has recently been recognized as a proliferative disease. Over the last 15 years great advances have been achieved in understanding the complex mechanisms of LAM on a molecular, cellular and tissue level. This has resulted in an important game changing therapy that can halt the progress of this disease in many patients. Since then additional therapeutic strategies that improve on mTOR inhibition alone have been developed. These strategies aim at eliminating the mutated TSC2−/− cells by reducing autophagy and antiapoptotic effects that also result from mTOR inhibition. Adding these newer strategies on top of mTOR inhibition may come closer to a state of healing LAM. This article presents the most important developments in this area.

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Authors and Affiliations

  1. Abteilung Pneumologie, Universitätsklinikum Leipzig AöR, Liebigstr. 20, Haus 4, 04103, Leipzig, Deutschland

    H. Wirtz

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  1. H. Wirtz
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Correspondence to H. Wirtz.

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Interessenkonflikt

H. Wirtz hat ein Vortragshonorar von Pfizer (Hersteller von Sirolimus) erhalten.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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U. Costabel, Essen

H. Wirtz, Leipzig

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Wirtz, H. LAM: Neue Therapien – neue Chancen?. Pneumologe 13, 13–19 (2016). https://doi.org/10.1007/s10405-015-0020-3

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