S3-Leitlinie: Lungenerkrankung bei Mukoviszidose – Modul 2: Diagnostik und Therapie bei der chronischen Infektion mit Pseudomonas aeruginosa

Zusammenfassung

Mukoviszidose (Cystic Fibrosis, CF) ist die häufigste, autosomal-rezessiv vererbte Multisystemerkrankung. In Deutschland sind ca. 8000 Menschen betroffen.

Die Erkrankung wird durch Mutationen im Cystic Fibrosis Transmembrane Conductance Regulator (CFTR-) Gen verursacht; diese führen zu einer Fehlfunktion des Chloridkanals CFTR. Dadurch kommt es in den Atemwegen zu einer unzureichenden Hydrierung des epithelialen Flüssigkeitsfilms und somit zu einer chronischen Inflammation.

Rezidivierende Infektionen der Atemwege sowie pulmonale Exazerbationen der Lunge führen im Verlauf zu zunehmender Inflammation, pulmonaler Fibrose und fortschreitender Lungendestruktion bis hin zur respiratorischen Globalinsuffizienz, die für über 90 % der Mortalität verantwortlich ist.

Das Ziel der medikamentösen Therapie ist die pulmonale Inflammation und v. a. die Infektion der Atemwege zu reduzieren.

Der Kolonisation und chronischen Infektion mit Pseudomonas aeruginosa (Pa) kommt die größte Bedeutung zu. Diese führt zu weiterem Verlust an Lungenfunktion.

Für die medikamentöse Therapie der chronischen Pa-Infektion stehen viele unterschiedliche Therapieoptionen zur Verfügung.

Mit dieser S3-Leitlinie wird eine einheitliche Definition für die chronische Pa-Infektion implementiert sowie eine evidenzbasierte Diagnostik und Therapie dargelegt, um eine Orientierung bei der individuellen Therapieentscheidung zu geben.

Abstract

Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the epithelial lining fluid which leads to chronic inflammation of the airways.

Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90 % of patients.

The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection.

Pseudomonas aeruginosa (Pa) is the most relevant pathogen in the course of CF lung disease. Colonization and chronic infection are leading to additional loss of pulmonary function. There are many possibilities to treat Pa-infection.

This is a S3-clinical guideline which implements a definition for chronic Pa-infection and demonstrates evidence-based diagnostic methods and medical treatment for Pa-infection in order to give guidance for individual treatment options.

^a Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP), federführende Fachgesellschaft

^b Gesellschaft für Pädiatrische Pneumologie e. V. (GPP), federführende Fachgesellschaft

^c Paul-Ehrlich Gesellschaft für Chemotherapie e. V. (PEG)

^d Deutsche Gesellschaft für Kinder- und Jugendmedizin e. V. (DGKJ)

^e Deutsche Gesellschaft für Hygiene und Mikrobiologie e. V. (DGHM)

^f Deutsche Gesellschaft für HNO-Heilkunde, Kopf- und Hals-Chirurgie e. V. (HNO)

^g Deutsche Gesellschaft für pädiatrische Infektiologie e. V. (DGPI)

^h Allianz Chronischer Seltener Erkrankungen (ACHSE) e. V.

ⁱ Österreichische Gesellschaft für Kinder- und Jugendheilkunde (ÖGKJ)

^j Deutsche Gesellschaft für Infektiologie e. V. (DGI)

^k Deutsche Röntgengesellschaft e. V. (DRG)

^l Deutscher Verband für Physiotherapie (ZVK) e. V.

^m Swiss Working Group for Cystic Fibrosis (SWGCF)

ⁿ Mukoviszidose e. V.

^o Mukoviszidose Institut gGmbH (MI)

^p Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e.V. (AWMF)

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