Pneumologie 2024; 78(11): 833-899
DOI: 10.1055/a-2311-9450
Leitlinie

Management erwachsener Patientinnen und Patienten mit Bronchiektasen-Erkrankung[*]

Eine S2k-Leitlinie der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP) (AWMF-Registernummer 020-030)Management of adult bronchiectasisConsensus-based Guidelines for the German Respiratory Society (DGP) e. V. (AWMF registration number 020-030)
Felix C. Ringshausen
 1   Klinik für Pneumologie und Infektiologie, Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
 2   Biomedical Research in End-Stage and Obstructive Lung Disease (BREATH), Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland
 3   European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Deutschland
,
Ingo Baumann
 4   Hals-, Nasen- und Ohrenklinik, Universitätsklinikum Heidelberg, Heidelberg, Deutschland
,
Andrés de Roux
 5   Pneumologische Praxis am Schloss Charlottenburg, Berlin, Deutschland
,
Sabine Dettmer
 2   Biomedical Research in End-Stage and Obstructive Lung Disease (BREATH), Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland
 6   Institut für Diagnostische und Interventionelle Radiologie, Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
,
Roland Diel
 7   Institut für Epidemiologie, Universitätsklinikum Schleswig-Holstein (UKSH), Kiel, Deutschland; LungenClinic Grosshansdorf, Airway Research Center North (ARCN), Deutsches Zentrum für Lungenforschung (DZL), Grosshansdorf, Deutschland
,
Monika Eichinger
 8   Klinik für Diagnostische und Interventionelle Radiologie, Thoraxklinik am Universitätsklinikum Heidelberg, Heidelberg, Deutschland; Translational Lung Research Center Heidelberg (TLRC), Deutsches Zentrum für Lungenforschung (DZL), Heidelberg, Deutschland
,
Santiago Ewig
 9   Thoraxzentrum Ruhrgebiet, Kliniken für Pneumologie und Infektiologie, EVK Herne und Augusta-Kranken-Anstalt Bochum, Bochum, Deutschland
,
Holger Flick
10   Klinische Abteilung für Pulmonologie, Universitätsklinik für Innere Medizin, LKH-Univ. Klinikum Graz, Medizinische Universität Graz, Graz, Österreich
,
Leif Hanitsch
11   Institut für Medizinische Immunologie, Charité – Universitätsmedizin Berlin, Freie Universität Berlin und Humboldt-Universität zu Berlin, Berlin, Deutschland
,
Thomas Hillmann
12   Ruhrlandklinik, Westdeutsches Lungenzentrum am Universitätsklinikum Essen, Essen, Deutschland
,
Rembert Koczulla
13   Abteilung für Pneumologische Rehabilitation, Philipps Universität Marburg, Marburg, Deutschland
,
Michael Köhler
14   Deutsche Patientenliga Atemwegserkrankungen e. V.
,
Assen Koitschev
15   Klinik für Hals-, Nasen-, Ohrenkrankheiten, Klinikum Stuttgart – Olgahospital, Stuttgart, Deutschland
,
Christian Kugler
16   Abteilung Thoraxchirurgie, LungenClinic Grosshansdorf, Grosshansdorf, Deutschland
,
Thomas Nüßlein
17   Klinik für Kinder- und Jugendmedizin, Gemeinschaftsklinikum Mittelrhein gGmbH, Koblenz, Deutschland
,
Sebastian R. Ott
18   Pneumologie/Thoraxchirurgie, St. Claraspital AG, Basel; Universitätsklinik für Pneumologie, Allergologie und klinische Immunologie, Inselspital, Universitätsspital und Universität Bern, Bern, Schweiz
,
Isabell Pink
 1   Klinik für Pneumologie und Infektiologie, Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
 2   Biomedical Research in End-Stage and Obstructive Lung Disease (BREATH), Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland
 3   European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Deutschland
,
Mathias Pletz
19   Institut für Infektionsmedizin und Krankenhaushygiene, Universitätsklinikum Jena, Jena, Deutschland
,
Gernot Rohde
20   Pneumologie/Allergologie, Medizinische Klinik 1, Universitätsklinikum Frankfurt, Goethe-Universität, Frankfurt am Main, Deutschland
,
Ludwig Sedlacek
21   Institut für Medizinische Mikrobiologie und Krankenhaushygiene, Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
,
Hortense Slevogt
 1   Klinik für Pneumologie und Infektiologie, Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
 2   Biomedical Research in End-Stage and Obstructive Lung Disease (BREATH), Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland
22   Center for Individualised Infection Medicine, Hannover, Deutschland
,
Urte Sommerwerck
23   Klinik für Pneumologie, Allergologie, Schlaf- und Beatmungsmedizin, Cellitinnen-Severinsklösterchen Krankenhaus der Augustinerinnen, Köln, Deutschland
,
Sivagurunathan Sutharsan
24   Klinik für Pneumologie, Universitätsmedizin Essen – Ruhrlandklinik, Essen, Deutschland
,
Sönke von Weihe
16   Abteilung Thoraxchirurgie, LungenClinic Grosshansdorf, Grosshansdorf, Deutschland
,
Tobias Welte (†)
 1   Klinik für Pneumologie und Infektiologie, Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
 2   Biomedical Research in End-Stage and Obstructive Lung Disease (BREATH), Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland
 3   European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Deutschland
,
Michael Wilken
14   Deutsche Patientenliga Atemwegserkrankungen e. V.
,
Jessica Rademacher#
 1   Klinik für Pneumologie und Infektiologie, Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
 2   Biomedical Research in End-Stage and Obstructive Lung Disease (BREATH), Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland
 3   European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Deutschland
,
Pontus Mertsch#
25   Medizinische Klinik und Poliklinik V, Klinikum der Universität München (LMU), Comprehensive Pneumology Center (CPC), Deutsches Zentrum für Lungenforschung (DZL), München, Deutschland
,
Weitere beteiligte Wissenschaftliche Fachgesellschaften und Organisationen: Bundesverband der Pneumologen, Schlaf- und Beatmungsmediziner e. V. (BdP), Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V. (DGHNO-KHC), Deutsche Gesellschaft für Hygiene und Mikrobiologie e. V. (DGHM), Deutschen Gesellschaft für Immunologie e. V. (DGfI), Deutsche Gesellschaft für Infektiologie e. V. (DGI), Deutsche Gesellschaft für Rehabilitationswissenschaften e. V. (DGRW), Deutsche Gesellschaft für Thoraxchirurgie e. V. (DGT), Deutsche Deutsche Patientenliga Atemwegserkrankungen e. V. (DPLA), Deutsche Röntgen-Gesellschaft, Gesellschaft für Medizinische Radiologie e. V. (DRG), Deutscher Verband für Physiotherapie e. V. (ZVK), Gesellschaft für Pädiatrische Pneumologie e. V. (GPP), Deutsche Österreichische Gesellschaft für Pneumologie (ÖGP), Paul-Ehrlich Gesellschaft für Infektionstherapie e. V. (PEG), Schweizerische Gesellschaft für Pneumologie (SGP) › Author Affiliations

Zusammenfassung

Die Bronchiektasen-Erkrankung ist eine ätiologisch heterogene, chronische und oftmals progredient verlaufende Atemwegs- und Lungenerkrankung, die durch eine irreversible Erweiterung der Bronchien gekennzeichnet ist. Sie geht häufig mit einer erheblichen Symptomlast, multiplen Komplikationen sowie einer eingeschränkten Lebensqualität einher. Seit mehreren Jahren ist weltweit eine deutliche Zunahme der Prävalenz der Bronchiektasen-Erkrankung mit einer relevanten ökonomischen Belastung der Gesundheitssysteme zu beobachten. Die vorliegende konsensusbasierte Leitlinie ist die erste deutschsprachige Leitlinie, die das Management der Bronchiektasen-Erkrankung bei Erwachsenen behandelt. Die Leitlinie betont die Wichtigkeit der thorakalen Bildgebung mittels CT zur Diagnose und Differenzierung der Bronchiektasen sowie die Bedeutung der Ätiologie zur Festlegung der Therapieansätze. Es werden sowohl nicht-medikamentöse als auch medikamentöse Therapien ausführlich erörtert. Zu den nicht-medikamentösen Maßnahmen gehören Raucherentwöhnung, Physiotherapie, körperliches Training, Rehabilitation, nichtinvasive Beatmung, Thoraxchirurgie und Lungentransplantation. Bei den medikamentösen Therapien wird besonders auf die langfristige Anwendung von Mukolytika, Bronchodilatatoren, antiinflammatorischen Medikamenten und Antibiotika eingegangen. Darüber hinaus geht die Leitlinie auf die Herausforderungen und Strategien bei der Behandlung einer oberen Atemwegsbeteiligung, von Komorbiditäten und Exazerbationen sowie die sozialmedizinischen Aspekte und das Schwerbehindertenrecht ein. Zudem wird die Bedeutung der Patientenaufklärung und des Selbstmanagements hervorgehoben. Abschließend werden spezielle Lebensphasen wie Transition, Kinderwunsch, Schwangerschaft und Elternschaft sowie Palliativmedizin behandelt. Die Leitlinie zielt darauf ab, eine umfassende, konsensusbasierte und patientenzentrierte Versorgung zu gewährleisten, wobei individuelle Risiken und Bedürfnisse berücksichtigt werden.

Abstract

Bronchiectasis is an etiologically heterogeneous, chronic, and often progressive respiratory disease characterized by irreversible bronchial dilation. It is frequently associated with significant symptom burden, multiple complications, and reduced quality of life. For several years, there has been a marked global increase in the prevalence of bronchiectasis, which is linked to a substantial economic burden on healthcare systems. This consensus-based guideline is the first German-language guideline addressing the management of bronchiectasis in adults. The guideline emphasizes the importance of thoracic imaging using CT for diagnosis and differentiation of bronchiectasis and highlights the significance of etiology in determining treatment approaches. Both non-drug and drug treatments are comprehensively covered. Non-pharmacological measures include smoking cessation, physiotherapy, physical training, rehabilitation, non-invasive ventilation, thoracic surgery, and lung transplantation. Pharmacological treatments focus on the long-term use of mucolytics, bronchodilators, anti-inflammatory medications, and antibiotics. Additionally, the guideline covers the challenges and strategies for managing upper airway involvement, comorbidities, and exacerbations, as well as socio-medical aspects and disability rights. The importance of patient education and self-management is also emphasized. Finally, the guideline addresses special life stages such as transition, family planning, pregnancy and parenthood, and palliative care. The aim is to ensure comprehensive, consensus-based, and patient-centered care, taking into account individual risks and needs.

* Verabschiedet von den Vorständen der beteiligten Fachgesellschaften und Organisationen am 03.04.2024


# Geteilte Letztautorenschaft




Publication History

Article published online:
08 November 2024

© 2024. Thieme. All rights reserved.

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