S3-Leitlinie: Lungenerkrankung bei Mukoviszidose – Pseudomonas aeruginosa

Carsten Schwarz; Jutta Bend; Helge Hebestreit; Michael Hogardt; Christian Hügel; Stephan Illing; Jochen G. Mainz; Ernst Rietschel; Sebastian Schmidt; Bernhard Schulte-Hubbert; Helmut Sitter; Marc Oliver Wielpütz; Jutta Hammermann; Ingo Baumann; Frank Brunsmann; Doris Dieninghoff; Ernst Eber; Helmut Ellemunter; Patience Eschenhagen; Caroline Evers; Saskia Gruber; Assen Koitschev; Julia Ley-Zaporozhan; Uta Düesberg; Hans-Joachim Mentzel; Thomas Nüßlein; Felix C. Ringshausen; Ludwig Sedlacek; Christina Smaczny; Olaf Sommerburg; Sivagurunathan Sutharsan; Ralf-Peter Vonberg; Ann-Katrin Weber; Jovita Zerlik

doi:10.1055/a-2182-1907

Pneumologie, Table of Contents

Pneumologie 2024; 78(06): 367-399
DOI: 10.1055/a-2182-1907

Leitlinie

S3-Leitlinie: Lungenerkrankung bei Mukoviszidose – Pseudomonas aeruginosa

CF Lung Disease – a German S3 Guideline: Pseudomonas aeruginosa

Carsten Schwarz^a

¹Klinikum Westbrandenburg GmbH, Standort Potsdam, Deutschland

,

Jutta Bend^b

²Mukoviszidose Institut gGmbH, Bonn, Deutschland

,

Helge Hebestreit^c

³Universitäts-Kinderklinik Würzburg, Deutschland

,

Michael Hogardt^d

⁴Klinikum der Johann Wolfgang Goethe-Universität Frankfurt am Main, Institut für Medizinische Mikrobiologie und Krankenhaushygiene, Frankfurt, Deutschland

,

Christian Hügel

⁵Klinikum der Johann Wolfgang Goethe-Universität Frankfurt am Main, Deutschland

,

Stephan Illing

⁶Nachsorgeklinik Tannheim, VS-Tannheim, Deutschland

,

Jochen G. Mainz

⁷Klinikum Westbrandenburg, Standort Brandenburg an der Havel, Universitätsklinikum der Medizinischen Hochschule Brandenburg (MHB), Brandenburg an der Havel, Deutschland

,

Ernst Rietschel^c

⁸Medizinische Fakultät der Universität zu Köln, Mukoviszidose-Zentrum, Klinik und Poliklinik für Kinder- und Jugendmedizin, Köln, Deutschland

,

Sebastian Schmidt^e

⁹Ernst-Moritz-Arndt Universität Greifswald, Kinderpoliklinik, Allgemeine Pädiatrie, Greifswald, Deutschland

,

Bernhard Schulte-Hubbert

¹⁰Medizinische Klinik und Poliklinik I, Pneumologie, Universitätsklinikum Dresden, Deutschland

,

Helmut Sitter

¹¹Philipps-Universität Marburg, Institut für theoretische Medizin, Marburg, Deutschland

,

Marc Oliver Wielpütz^f

¹²Universitätsklinikum Heidelberg, Klinik für Diagnostische und Interventionelle Radiologie, Heidelberg, Deutschland

,

Jutta Hammermann^g

¹³Universitäts-Mukoviszidose-Zentrum „Christiane Herzog“, Dresden, Deutschland

,

Ingo Baumann^h

¹⁴Universität Heidelberg, Hals-Nasen-Ohrenklinik, Heidelberg, Deutschland

,

Frank Brunsmannⁱ

¹⁵Allianz Chronischer Seltener Erkrankungen (ACHSE) e. V., Deutschland (Patient*innenvertreter)

,

Doris Dieninghoff

¹⁶Kliniken der Stadt Köln, Lungenklinik Merheim, Köln, Deutschland

,

Ernst Eber^j

¹⁷Medizinische Universität Graz, Univ. Klinik für Kinder- und Jugendheilkunde, Klinische Abteilung für Pädiatrische Pulmonologie und Allergologie, Graz, Österreich

,

Helmut Ellemunter^k

¹⁸Tirolkliniken GmbH, Department für Kinderheilkunde, Pädiatrie III, Innsbruck, Österreich

,

Patience Eschenhagen

¹Klinikum Westbrandenburg GmbH, Standort Potsdam, Deutschland

,

Caroline Evers^l

¹⁹Hamburg (Patient*innenvertreterin), Deutschland

,

Saskia Gruber

²⁰Medizinische Universität Wien, Universitätsklinik für Kinder- und Jugendheilkunde, Wien, Österreich

,

Assen Koitschev^h

²¹Klinikum Stuttgart – Standort Olgahospital, Klinik für Hals-Nasen-Ohrenkrankheiten, Stuttgart, Deutschland

,

Julia Ley-Zaporozhan^f

²²Klinik und Poliklinik für Radiologie, Kinderradiologie, LMU München, Deutschland

,

Uta Düesberg^b

²Mukoviszidose Institut gGmbH, Bonn, Deutschland

,

Hans-Joachim Mentzel^m

²³Universitätsklinikum Jena, Sektion Kinderradiologie, Institut für Diagnostische und Interventionelle Radiologie, Jena, Deutschland

,

Thomas Nüßlein

²⁴Gemeinschaftsklinikum Mittelrhein, Klinik für Kinder- und Jugendmedizin Koblenz und Mayen, Koblenz, Deutschland

,

Felix C. Ringshausenⁿ

²⁵Medizinische Hochschule Hannover, Klinik für Pneumologie und Infektiologie und Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland

,

Ludwig Sedlacek

²⁶Medizinische Hochschule Hannover, Institut für Medizinische Mikrobiologie und Krankenhaushygiene, Hannover, Deutschland

,

Christina Smaczny

⁵Klinikum der Johann Wolfgang Goethe-Universität Frankfurt am Main, Deutschland

,

Olaf Sommerburg

²⁷Universitätsklinikum Heidelberg, Sektion Pädiatrische Pneumologie, Allergologie und Mukoviszidose-Zentrum, Heidelberg, Deutschland

,

Sivagurunathan Sutharsan

²⁸Universitätsmedizin Essen Ruhrlandklinik, Pneumologie, Essen, Deutschland

,

Ralf-Peter Vonberg

²⁹Medizinische Hochschule Hannover, Institut für Medizinische Mikrobiologie und Krankenhaushygiene, Hannover, Deutschland

,

Ann-Katrin Weber^b

²Mukoviszidose Institut gGmbH, Bonn, Deutschland

,

Jovita Zerlik^o

³⁰Altonaer Kinderkrankenhaus gGmbH, Abteilung Physiotherapie, Hamburg, Deutschland

› Author Affiliations

Abstract

Zusammenfassung

Mukoviszidose (Cystic Fibrosis, CF) ist die häufigste autosomal-rezessiv vererbte Multisystemerkrankung. In Deutschland sind mind. 8000 Menschen betroffen. Die Erkrankung wird durch Mutationen im Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Gen verursacht, welche zu einer Fehlfunktion des Chloridkanals CFTR führen. Dadurch kommt es in den Atemwegen zu einer unzureichenden Hydrierung des epithelialen Flüssigkeitsfilms und somit zu einer Reduktion der mukoziliären Clearance.

Auch wenn seit einigen Jahren mit der CFTR-Modulatortherapie eine hochwirksame kausale CF-Therapie zur Verfügung steht und die Patienten größtenteils das höhere Erwachsenenalter erreichen, treten rezidivierende und chronische Infektionen der Atemwege sowie pulmonale Exazerbationen weiterhin auf. Im Erwachsenenalter zeigt sich v. a. die Kolonisation und chronische Infektion mit Pseudomonas aeruginosa (PA), die zu weiterem Verlust an Lungenfunktion führt. Für die medikamentöse Therapie der chronischen PA-Infektion stehen viele unterschiedliche Therapieoptionen zur Verfügung.

Mit dieser S3-Leitlinie wird eine evidenzbasierte Diagnostik und Therapie der PA-Infektion dargelegt, um eine Orientierung bei der individuellen Therapieentscheidung zu geben.

Abstract

Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. 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 airway epithelial lining fluid which leads to reduction of the mucociliary clearance.

Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, Pseudomonas aeruginosa (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung 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 in order to give guidance for individual treatment options.

Full Text

References

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