Zentralbl Chir 2021; 146(03): 346-358
DOI: 10.1055/a-1361-2252
CME-Fortbildung

Interdisziplinäre Behandlung der Non-Cystic-Fibrosis-Bronchiektasie – State of the Art

Multidisciplinary Management of Non-cystic-fibrosis Bronchiectasis – State of the Art
Pontus Mertsch
,
Hayan Merhej
,
Patrick Zardo

Zusamenfassung

Bronchiektasien, die nicht durch eine Zystische Fibrose bedingt sind, sind eine ätiologisch äußerst heterogene Erkrankung. Dieser Beitrag gibt einen Überblick über die Ursachen, das diagnostische Vorgehen und die therapeutischen Optionen bei dieser nicht seltenen Lungenerkrankung.

Abstract

Bronchiectasis is a mostly irreversible bronchial dilatation induced by a destruction of elastic and muscular fibers of the bronchial wall. Radiological criteria of bronchiectasis are met, when the inner diameter of the bronchial wall surpasses the outer diameter of the accompanying pulmonary artery. Its incidence increases with age, even though it often lacks true clinical signs of disease. Only when it is accompanied by cough, expectorations and recurring bronchopulmonary infections, it can be considered a true bronchiectatic disease. Cystic fibrosis (CF) is one of its preeminent triggers, but certainly plays a particular role in this entity, which is why the terminus of “non-CF-bronchiectasis” was coined in the first place.

Multidisciplinary management consists in extensive diagnostic work-up, treatment of potential triggers of bronchiectasis and supportive care in form of vaccination programs, secretolysis and pulmonary rehabilitation, as well as antibiotic treatment of pulmonary exacerbations.

Surgical treatment has to be considered a last resort in case of hemoptysis, recurring severe pneumonia or secondary aspergilloma with complete resection of all pathological findings, ideally by minimally-invasive approach.

Kernaussagen
  • Trotz der insgesamt zunehmenden Inzidenz von Bronchiektasen in den westlichen Industrienationen haben die schweren, postinfektiösen Fälle durch gezielte Impfkampagnen und adäquates antibiotisches Management deutlich abgenommen.

  • Aufgrund der Vielzahl an möglichen Ursachen, welche zur Entstehung von Bronchiektasen führen, und der Überlappung mit anderen pneumologischen Krankheitsbildern werden Diagnostik und Behandlung zunehmend komplex.

  • Dank einer Vielzahl unterschiedlicher und komplementärer konservativer Therapieverfahren ist eine chirurgische Versorgung nur selten notwendig.

  • Sollte dies doch der Fall sein, kann diese an entsprechenden Zentren mit hoher Sicherheit oftmals in minimalinvasiver Form erfolgen.

  • Ziel ist in diesen Situationen eine vollständige Resektion des betroffenen Areals unter optimalem Erhalt des übrigen Parenchyms.



Publication History

Article published online:
21 June 2021

© 2021. Thieme. All rights reserved.

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  • Literatur

  • 1 Ringshausen FC, Rademacher J, Pink I. et al. Increasing bronchiectasis prevalence in Germany, 2009–2017: a population-based cohort study. Eur Respir J 2019; 54: 1900499
  • 2 Diel R, Chalmers JD, Rabe KF. et al. Economic burden of bronchiectasis in Germany. Eur Respir J 2018; 53: 1802033
  • 3 Chalmers J, Polverino E, Aliberti S. Bronchiectasis. The EMBARC Manual. Berlin: Springer; 2018
  • 4 Araújo D, Shteinberg M, Aliberti S. et al. Standardised classification of the aetiology of bronchiectasis using an objective algorithm. Eur Respir J 2017; 50: 1701289
  • 5 Polverino E, Goeminne PC, McDonnell MJ. et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J 2017; 50: 1700629
  • 6 Bilton D, Tino G, Barker AF. et al. Inhaled mannitol for non-cystic fibrosis bronchiectasis: a randomised, controlled trial. Thorax 2014; 69: 1073-1079
  • 7 OʼDonnell AE, Barker AF, Ilowite JS. et al. Treatment of Idiopathic Bronchiectasis With Aerosolized Recombinant Human DNase I. Chest 1998; 113: 1329-1334
  • 8 Boaventura R, Sibila O, Agusti A. et al. Treatable traits in bronchiectasis. Eur Respir J 2018; 52: 1801269
  • 9 Rademacher J, Konwert S, Fuge J. et al. Anti-IL5 and anti-IL5Rα therapy for clinically significant bronchiectasis with eosinophilic endotype: a case series. Eur Respir J 2019; 1901333
  • 10 Sibila O, Laserna E, Shoemark A. et al. Airway Bacterial Load and Inhaled Antibiotic Response in Bronchiectasis. Am J Resp Crit Care 2019; 200: 33-41
  • 11 Middleton PG, Mall MA, Dřevínek P. et al. Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. N Engl J Med 2019; 381: 1809-1819
  • 12 Houtmeyers E, Gosselink R, Gayan-Ramirez G. et al. Regulation of mucociliary clearance in health and disease. Eur Respir J 1999; 13: 1177-1188
  • 13 Muñoz G, Gracia J de Buxó M. et al. Long-term benefits of airway clearance in bronchiectasis: a randomised placebo-controlled trial. Eur Respir J 2018; 51: 1701926
  • 14 Herrero-Cortina B, Vilaró J, Martí D. et al. Short-term effects of three slow expiratory airway clearance techniques in patients with bronchiectasis: a randomised crossover trial. Physiotherapy 2016; 102: 357-364
  • 15 Syed N, Maiya AG, Kumar TS. Active Cycles of Breathing Technique (ACBT) versus conventional chest physical therapy on airway clearance in bronchiectasis – A crossover trial. Adv Physiotherapy 2009; 11: 193-198
  • 16 Murray MP, Pentland JL, Hill AT. A randomised crossover trial of chest physiotherapy in non-cystic fibrosis bronchiectasis. Eur Respir J 2009; 34: 1086-1092
  • 17 Nicolson CHH, Stirling RG, Borg BM. et al. The long term effect of inhaled hypertonic saline 6 % in non-cystic fibrosis bronchiectasis. Respir Med 2012; 106: 661-667
  • 18 Kellett F, Robert NM. Nebulised 7 % hypertonic saline improves lung function and quality of life in bronchiectasis. Respir Med 2011; 105: 1831-1835
  • 19 Lee AL, Hill CJ, Cecins N. et al. The short and long term effects of exercise training in non-cystic fibrosis bronchiectasis – a randomised controlled trial. Respir Res 2014; 15: 44
  • 20 Lee AL, Hill CJ, McDonald CF. et al. Pulmonary Rehabilitation in Individuals With Non–Cystic Fibrosis Bronchiectasis: A Systematic Review. Arch Phys Med Rehab 2017; 98: 774-782.e1
  • 21 Mandal P, Sidhu MK, Kope L. et al. A pilot study of pulmonary rehabilitation and chest physiotherapy versus chest physiotherapy alone in bronchiectasis. Respir Med 2012; 106: 1647-1654
  • 22 Hill AT, Haworth CS, Aliberti S. et al. Pulmonary exacerbation in adults with bronchiectasis: a consensus definition for clinical research. Eur Respir J 2017; 49: 1700051
  • 23 Chalmers JD, Goeminne P, Aliberti S. et al. The Bronchiectasis Severity Index. An International Derivation and Validation Study. Am J Respir Crit Care Med 2014; 189: 576-585
  • 24 Aliberti S, Lonni S, Dore S. et al. Clinical phenotypes in adult patients with bronchiectasis. Eur Respir J 2016; 47: 1113-1122
  • 25 McDonnell MJ, Jary HR, Perry A. et al. Non cystic fibrosis bronchiectasis: A longitudinal retrospective observational cohort study of Pseudomonas persistence and resistance. Respir Med 2015; 109: 716-726
  • 26 Pieters A, Bakker M, Hoek RAS. et al. Predicting factors for chronic colonization of Pseudomonas aeruginosa in bronchiectasis. Eur J Clin Microbiol 2019; 38: 2299-2304
  • 27 Polverino E, Rosales-Mayor E, Benegas M. et al. Pneumonic and non-pneumonic exacerbations in bronchiectasis: Clinical and microbiological differences. J Infection 2018; 77: 99-106
  • 28 Ewig S, Höffken G, Kern W. et al. Behandlung von erwachsenen Patienten mit ambulant erworbener Pneumonie und Prävention – Update 2016*. Pneumologie 2016; 70: 151-200
  • 29 Hill AT, Sullivan AL, Chalmers JD. et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax 2019; 74: 1
  • 30 Mertsch P, Pink I, Rademacher J. et al. Eradication of Pseudomonas aeruginosa within the baseline dataset of the German Bronchiectasis Registry PROGNOSIS. Eur Respir J 2020; 56 (Suppl. 64) 2366
  • 31 Vallières E, Tumelty K, Tunney MM. et al. Efficacy of Pseudomonas aeruginosa eradication regimens in bronchiectasis. Eur Respir J 2017; 49: 1600851
  • 32 Cramer CL, Patterson A, Alchakaki A. et al. Immunomodulatory indications of azithromycin in respiratory disease: a concise review for the clinician. Postgrad Med 2017; 129: 493-499
  • 33 Altenburg J, Graaff CS de Stienstra Y. et al. Effect of azithromycin maintenance treatment on infectious exacerbations among patients with non-cystic fibrosis bronchiectasis: the BAT randomized controlled trial. JAMA 2013; 309: 1251-1259
  • 34 Wong C, Jayaram L, Karalus N. et al. Azithromycin for prevention of exacerbations in non-cystic fibrosis bronchiectasis (EMBRACE): a randomised, double-blind, placebo-controlled trial. Lancet 2012; 380: 660-667
  • 35 Serisier DJ, Martin ML, McGuckin MA. et al. Effect of long-term, low-dose erythromycin on pulmonary exacerbations among patients with non-cystic fibrosis bronchiectasis: the BLESS randomized controlled trial. JAMA 2013; 309: 1260-1267
  • 36 Daley CL, Iaccarino JM, Lange C. et al. Treatment of Nontuberculous Mycobacterial Pulmonary Disease: An Official ATS/ERS/ESCMID/IDSA Clinical Practice Guideline. Clin Infect Dis 2020; 71: 905-913
  • 37 Chalmers JD, Smith MP, McHugh BJ. et al. Short- and Long-Term Antibiotic Treatment Reduces Airway and Systemic Inflammation in Non-Cystic Fibrosis Bronchiectasis. Am J Resp Crit Care 2012; 186: 657-665
  • 38 Haworth CS, Foweraker JE, Wilkinson P. et al. Inhaled colistin in patients with bronchiectasis and chronic Pseudomonas aeruginosa infection. Am J Resp Crit Care 2014; 189: 975-982
  • 39 Haworth CS, Bilton D, Chalmers JD. et al. Inhaled liposomal ciprofloxacin in patients with non-cystic fibrosis bronchiectasis and chronic lung infection with Pseudomonas aeruginosa (ORBIT-3 and ORBIT-4): two phase 3, randomised controlled trials. Lancet Respir Med 2019; 7: 213-226
  • 40 Barker AF, OʼDonnell AE, Flume P. et al. Aztreonam for inhalation solution in patients with non-cystic fibrosis bronchiectasis (AIR-BX1 and AIR-BX2): two randomised double-blind, placebo-controlled phase 3 trials. Lancet Respir Med 2014; 2: 738-749
  • 41 Soyza AD, Aksamit T, Bandel TJJ. et al. RESPIRE 1: a phase III placebo-controlled randomised trial of ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis. Eur Respir J 2018; 51: 1702052
  • 42 Aksamit T, Soyza AD, Bandel TJJ. et al. RESPIRE 2: a phase III placebo-controlled randomised trial of ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis. Eur Respir J 2018; 51: 1702053
  • 43 Murray MP, Govan JRW, Doherty CJ. et al. A Randomized Controlled Trial of Nebulized Gentamicin in Non-Cystic Fibrosis Bronchiectasis. Am J Resp Crit Care 2011; 183: 491-499
  • 44 Barker AF, Couch L, Fiel SB. et al. Tobramycin Solution for Inhalation Reduces Sputum Pseudomonas aeruginosa Density in Bronchiectasis. Am J Resp Crit Care 2000; 162: 481-485
  • 45 Elborn JS, Geller DE, Conrad D. et al. A phase 3, open-label, randomized trial to evaluate the safety and efficacy of levofloxacin inhalation solution (APT-1026) versus tobramycin inhalation solution in stable cystic fibrosis patients. J Cyst Fibros 2015; 14: 507-514
  • 46 Flume PA, VanDevanter DR, Morgan EE. et al. A phase 3, multi-center, multinational, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of levofloxacin inhalation solution (APT-1026) in stable cystic fibrosis patients. J Cyst Fibros 2016; 15: 495-502
  • 47 Griffith DE, Eagle G, Thomson R. et al. Amikacin Liposome Inhalation Suspension for Treatment-Refractory Lung Disease Caused by Mycobacterium avium Complex (CONVERT). A Prospective, Open-Label, Randomized Study. Am J Respir Crit Care Med 2018; 198: 1559-1569
  • 48 Brodt A, Stovold E, Zhang L. Inhaled antibiotics for stable non-cystic fibrosis bronchiectasis: a systematic review. Eur Respir J 2014; 44: 382-393
  • 49 Laska IF, Crichton ML, Shoemark A. et al. The efficacy and safety of inhaled antibiotics for the treatment of bronchiectasis in adults: a systematic review and meta-analysis. Lancet Respir Med 2019; 7: 855-869
  • 50 Chalmers JD, Haworth CS, Metersky ML. et al. Phase 2 Trial of the DPP-1 Inhibitor Brensocatib in Bronchiectasis. N Engl J Med 2020; 383: 2127-2137
  • 51 Sehitogullari A, Bilici S, Sayir F. et al. A long term study assessing the factors influencing survival and morbidity in the surgical management of bronchiectasis. J Cardiothorac Surg 2011; 6: 161
  • 52 Balkanli K, Genc O, Dakak M. et al. Surgical Management of bronchiectasis: analysis and short-term results in 238 patients. Eur J Cardiothorac Surg 2003; 24: 699-702
  • 53 Cook JC, Currie DC, Morgan AD. et al. Role of computed tomography in diagnosis of bronchiectasis. Thorax 1987; 42: 272-277
  • 54 Lee JH, Kim YK, Kwag HJ. et al. Relationships between high-resolution computed tomography, lung function and bacteriology in stable bronchiectasis. J Korean Med Sci 2004; 19: 62-68
  • 55 Fujimoto T, Hillejan L, Stamatis G. Current strategy for surgical management of bronchiectasis. Ann Thorac Surg 2001; 72: 1711-1715
  • 56 Deslauriers J, Goulet S, Francois B. et al. Surgical Treatment of Bronchiectasis and Broncholithiasis. In: Franco LF, Putnam JB. eds. Advanced Therapy in thoracic Surgery. Hamilton, ON: Decker; 1998: 300-309