BRONCHIECTASIS and PRIMARY CILIARY DYSKINESIA
BTS QUALITY STANDARDS (2012)
www.brit-thoracic.org.uk/Portals/0/Guidelines/Bronchiectasis/244457_BTS_Quality_Standards_Bronchiectasis.pdf

BTS GUIDELINES (2010)
www.brit-thoracic.org.uk/Portals/0/Guidelines/Bronchiectasis/non-CF-Bronchiectasis-guideline.pdf

The June 2012 issue of Clinics in Chest Medicine is devoted to bronchiectasis (volume 33, issue 2).
www.chestmed.theclinics.com/issues?issue_key=S0272-5231%2811%29X0007-2


Angrill J, Agusti C, de Celis R (2002) Bacterial colonization in patients with bronchiectasis. Thorax, 57, 15-19.
thorax.bmjjournals.com/cgi/content/full/57/1/15?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=Angrill+J&andorexactfulltext=and&searchid=1121537321503_1039&stored_search=&FIRSTINDEX=0&sortspec=relevance&volume=57&firstpage=15&resourcetype=1&journalcode=thoraxjnl
• periodic sputum culture needed to identify bugs, which colonise distal airways of 60% of patients

Barker AF (2000) Tobramycin solution for inhalation reduces sputum pseudomonas aeruginosa density in bronchiectasis. Am J Respir Crit Care Med, 62, 481-485
ajrccm.atsjournals.org/cgi/content/full/162/2/481
• inhaled tobramycin reduces sputum P. aeruginosa density

Barker AF (2002) Bronchiectasis. New Eng J Med, 346, 1383-93
• comprehensive information

Behan L, Dimitrov BD, Kuehni CE et al (2016) PICADAR: a diagnostic predictive tool for primary ciliary dyskinesia. Eur Respir J; 47(4):1103-12. doi: 10.1183/13993003.01551-2015
erj.ersjournals.com/content/47/4/1103

Benhamou D, Muir JF, Radpaud C (1997) Long-term efficiency of home nasal mask ventilation in patients with diffuse bronchiectasis and severe chronic respiratory failure. Chest, 112, 1259-66
www.chestjournal.org/cgi/reprint/112/5/1259?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=Benhamou+D&andorexactfulltext=and&searchid=1121540953305_3840&stored_search=&FIRSTINDEX=0&sortspec=relevance&resourcetype=1&journalcode=chest
• NIV has shown reduced hospitalizations and improved functional status in diffuse disease

Bradley J, Moran F (2006) Pulmonary rehabilitation improves exercise tolerance in patients with bronchiectasis. Austr J Physiother, 52, 65.
• commentary on article describing improved exercise tolerance in people with bronchiectasis

Bulcun E, Arslan M, Ekici A et al (2015) Quality of life and bronchial hyper-responsiveness in subjects with bronchiectasis: validation of the seattle obstructive lung disease questionnaire in bronchiectasis. Respir Care; 60(11):1616-1623

Carotenuto M (2013) Psychological, cognitive and maternal stress assessment in children with primary ciliary dyskinesia. World J Pediatr; 9(4):312-7

Chatzidionisyou A, Catrina AI (2016) The lung in rheumatoid arthritis, cause or consequence? Curr Opin Rheumatol; 28(1):76-82. doi: 10.1097/BOR.0000000000000238
www.ncbi.nlm.nih.gov/pubmed/26599384
• association with RA

Cobanoglu U, Yalcinkaya I, Er M et al 2011 Surgery for bronchiectasis: The effect of morphological types to prognosis. Ann Thorac Med, 6, 1, 25–32
www.ncbi.nlm.nih.gov/pmc/articles/pmc3023867/?tool=pmcentrez

Çiftçi F (2013) Primary Ciliary Dyskinesia. Respir Care, 58(7):e99-e103

Dentice R (2009) Airway clearance physiotherapy improves quality of life in people with bronchiectasis. Austr J Physiotherapy, 55, 4, 285
proquest.umi.com/pqdweb?index=4&did=1936835881&SrchMode=3&sid=1&Fmt=6&VInst=PROD&VType=PQD&RQT=309&VName=PQD&TS=1269187944&clientId=64672&aid=2
• Acapella b.d. improves quality of life and exercise capacity

Dhillon SS (2000) Lady Windermere syndrome: middle lobe bronchiectasis and mycobacterium avium complex infection due to voluntary cough suppression. Clin Infectious Dis, 30, 3, 572-575
• cough suppression should not be used for social reasons

Du Q, Jin J, Liu X, Sun Y (2016) Bronchiectasis as a comorbidity of chronic obstructive pulmonary disease: a systematic review and meta-analysis. PLoS One; 11(3):e0150532. doi: 10.1371/journal.pone.0150532
journals.plos.org/plosone/article?id=10.1371/journal.pone.0150532

Eaton T, Young P, Zeng I, et al (2007) A randomized evaluation of the acute efficacy, acceptability and tolerability of flutter and active cycle of breathing with and without postural drainage in non-cystic fibrosis bronchiectasis. Chron Respir Dis, 4, 23–30
crd.sagepub.com/content/4/1/23.abstract?ijkey=18cc79bd2061f7685b648226fab353b3264eedfe&keytype2=tf_ipsecsha
• ACBT has shown superiority to the Flutter or postural drainage

Edwards EA, Narang I, Li A, et al (2004) HRCT lung abnormalities are not a surrogate for exercise limitation in bronchiectasis. Eur Respir J, 24, 538 - 44
erj.ersjournals.com/cgi/content/abstract/24/4/538
• high resolution computed tomography is not related to exercise capacity

Elkins MR, Anderson SD, Perry CP et al (2014) Inspiratory flows and volumes in subjects with non-CF bronchiectasis using a new dry powder inhaler device. Open Respir Med J; 8:8-13. doi: 10.2174/1874306401408010008

ElMaraachli W, Conrad DJ, Wang AC (2016) Using cystic fibrosis therapies for non-cystic fibrosis bronchiectasis. Clin Chest Med; 37(1):139-46. doi: 10.1016/j.ccm.2015.11.005

Feng W, Deng WW (1998) Short-term efficacy of RC-Cornet in bronchiectasis. Chest, 114, 320
• the Cornet decreases sputum viscosity

Figueiredo BC (2011) The role of macrolides in noncystic fibrosis bronchiectasis. Pulm Med; 751982 Article ID 751982. doi:10.1155/2011/751982

Flude LJ, Agent P, Bilton D (2012) Chest physiotherapy techniques in bronchiectasis. Clin Chest Med, 33, 2, 351-361

Fretzayas A, Moustaki M (2016) Clinical spectrum of primary ciliary dyskinesia in childhood. World J Clin Pediatr; 5(1):57-62. doi: 10.5409/wjcp.v5.i1.57
www.ncbi.nlm.nih.gov/pmc/articles/PMC4737693/?report=classic

Giilhan PY, Bulcun E, Giilhan M et al (2015) Low cognitive ability in subjects with bronchiectasis. Respir Care; 60(11):1610-1615

Goeminne P (2010) Non-cystic fibrosis bronchiectasis: diagnosis and management in 21st century. Postgrad Med J, 86, 493-501
pmj.bmj.com/content/86/1018/493.full
• patients need regular screening for infection
• inhaled mannitol may reduce the tenacity of mucus

Gould CM, Freeman AF, Olivier KN (2012) Genetic causes of bronchiectasis. Clin Chest Med; 33(2):249-63. doi: 10.1016/j.ccm.2012.03.002

Guan W-j, Gao Y-h, Gang X et al (2015) Inflammatory responses, spirometry, and quality of life in subjects with bronchiectasis exacerbations. Respir Care; 60(8):1180-1189

Guan WJ, Chen RC, Zhong NS (2016) The bronchiectasis severity index and FACED score for bronchiectasis. Eur Respir J; 47(2):382-4. doi: 10.1183/13993003.01717-2015
erj.ersjournals.com/content/47/2/382

Gülhan PY, Bulcun E, Giilhan M et al (2015) Low cognitive ability in subjects with bronchiectasis. Respir Care; 60(11):1610-1615

Hart A, Sugumar K, Milan SJ et al (2014) Inhaled hyperosmolar agents for bronchiectasis. Cochrane Database Syst Rev. May 12;5:CD002996. doi: 10.1002/14651858.CD002996.pub3

Hasani A, Chapman TH, McCool E et al (2008) Domiciliary humidification improves lung mucociliary clearance in patients with bronchiectasis. Chron Respir Dis, 5, 81-86

Horani A, Ferkol TW (2016) Primary ciliary dyskinesia and associated sensory ciliopathies. Expert Rev Respir Med; 28:1-8. [Epub ahead of print]

Hosie PH, Fitzgerald DA, Jaffe A et al (2015) Presentation of primary ciliary dyskinesia in children: 30 years' experience. J Paed Child Health; (51)7: 722–6

Judson MA, Chaudhry H, Compa DR et al (2014) A Delphi study of pharmacotherapy for noncystic fibrosis bronchiectasis. Am J Med Sci; 348(5):387-93. doi: 10.1097/MAJ.0000000000000312

Kapur N, Chang AB. Oral non steroid anti-inflammatories for children and adults with bronchiectasis. Cochrane Database of Systematic Reviews 2007, Issue 4. Art. No.: CD006427. DOI: 10.1002/14651858.CD006427.pub2
• anti inflammatory drugs may reduce the inflammatory cascade but have not been proven

King PT (2009) The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis, 4, 411-9
www.ncbi.nlm.nih.gov/pubmed/20037680?dopt=Abstract

King PT, Daviskas E (2010) Pathogenesis and diagnosis of bronchiectasis. Breathe, 6, 342-51
www.ers-education.org/pages/default.aspx?id=2041
• short and sweet, with pretty pictures.

Knowles MR, Daniels LA, Davis SD et al (2013) Primary ciliary dyskinesia. Recent advances in diagnostics, genetics, and characterization of clinical disease. Am J Respir Crit Care Med;188(8):913-22. doi: 10.1164/rccm.201301-0059CI

Koch MC, Pereira IA, Nobre LF et al (2016) Computed tomography of pulmonary changes in rheumatoid arthritis: carcinoembryonic antigen (CEA) as a marker of airway disease. Rheumatol Int; 36(4):531-9. doi: 10.1007/s00296-016-3438-y

Kuehni CE, Frischer T, Strippoli M-PF et al (2010) Factors influencing age at diagnosis of primary ciliary dyskinesia in European children. Eur Respir J, 36, 1248-1258, doi:10.1183/09031936.00001010

Lai M, Pifferi M, Bush A et al (2016) Gene editing of DNAH11 restores normal cilia motility in primary ciliary dyskinesia. J Med Genet; 53(4):242-9. doi: 10.1136/jmedgenet-2015-103539

Lasserson TJ, Holt K, Milan SJ, Greenstone M. Oral corticosteroids for bronchiectasis (stable and acute exacerbations). Cochrane Database of Systematic Reviews 2001, Issue 2. Art. No.: CD002162. DOI: 10.1002/14651858.CD002162
• steroids not proven to be effective

Lavery K, O'Neill B, Elborn JS, Reilly J, Bradley JM (2007) Self-management in bronchiectasis: the patients' perspective. Eur Respir J, 29, 541-7
erj.ersjournals.com/cgi/content/full/29/3/541
• reported benefits of physiotherapy include reduced shortness of breath, increased exercise capacity and better quality of life

Lee AL (2010) The effects of pulmonary rehabilitation in patients with non-cystic fibrosis bronchiectasis. BMC Pulm Med, 10, 5, doi:10.1186/1471-2466-10-5
www.biomedcentral.com/1471-2466/10/5

Lee AL, Button BM, Denehy L et al (2015) Exhaled breath condensate pepsin: potential noninvasive test for gastroesophageal reflux in COPD and bronchiectasis. Respir Care; 60(2):244-250

Leigh MW, Hazucha MJ, Chawla KK et al (2013) Standardizing nasal nitric oxide measurement as a test for primary ciliary dyskinesia. Ann Am Thorac Soc. 2013 Sep 11

Lin H, Cao Z, Zhao X et al (2016) Left middle lobectomy for bronchiectasis in a patient with Kartagener syndrome: a case report. J Cardiothorac Surg; 11(1):37. doi: 10.1186/s13019-016-0426-y

Lucas JS, Chetcuti P, Copeland F et al (2013) Overcoming challenges in the management of primary ciliary dyskinesia: The UK model. Paediatr Respir Rev; 15(2):142-145. doi: 10.1016/j.prrv.2013.04.007

Madsen A, Green K, Buchvald F et al (2013) Aerobic fitness in children and young adults with primary ciliary dyskinesia. PLoS One. 19;8(8):e71409. doi: 10.1371/journal.pone.0071409

Martinez-Garcia MA, Perpina-Tordera M, Roman-Sanchez P (2005) Quality of life determinants in patients with clinically stable bronchiectasis. Chest, 128, 739–45
chestjournal.chestpubs.org/content/128/2/739.full
• sputum and breathlessness are the main symptoms affecting quality of life

Mazières J (2003) Limited operation for severe multisegmental bilateral bronchiectasis.
Ann Thorac Surg; 75, 382-387
• limited surgery may be indicated in non-localized bilateral bronchiectasis with cystic and functionless areas

Nicolini A, Cardini F, Landucci N et al (2013) Effectiveness of treatment with high-frequency chest wall oscillation in patients with bronchiectasis. BMC Pulm Med; 4;13:21. doi: 10.1186/1471-2466-13-21

O’Callaghan C (2007) Diagnosing primary ciliary dyskinesia. Thorax, 62, 656-7

O'Donnell AE (2008) Bronchiectasis. Chest, 134, 4, 815-23
chestjournal.chestpubs.org/content/134/4/815.full
• pathology, diagnosis and treatment

O’Donnell AE (2011) Bronchiectasis in patients with COPD: a distinct COPD phenotype? Chest, 140, 5 1107-1108

Oktem S, Karadag B, Erdem E et al (2013) Sleep disordered breathing in patients with primary ciliary dyskinesia. Pediatr Pulmonol; 48(9):897-903. doi: 10.1002/ppul.22710

O'Leary CJ, Wilson CB, Hansell DM (2002) Relationship between psychological well-being and lung health status in patients with bronchiectasis. Respir Med, 96, 686–92
www.ncbi.nlm.nih.gov/pubmed/12243314?dopt=Abstract
• 34% of patients had elevated scores for anxiety and/or depression, with anxiety being the most common

O’Neill B, Bradley JM, McArdle (2002) The current physiotherapy management of patients with bronchiectasis. Int J Clin Pract, 56, 1, 34-5
www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11831830&dopt=Abstract

Ong HK, Lee AL, Hill CJ, Holland AE, Denehy L (2011) Effects of pulmonary rehabilitation in bronchiectasis. Chr Respir Dis, 8, 1, 21-31
• retrospective study supporting pulmonary rehabilitation for bronchiectasis patients

Paneroni M, Clini E, Simonelli C et al (2011) Safety and efficacy of short-term intrapulmonary percussive ventilation in patients with bronchiectasis. Respir Care; 56(7):984-8. doi: 10.4187/respcare.01098

Patel AS, Watkin G, Willig B et al (2011) Improvement in health status following cough-suppression physiotherapy for patients with chronic cough. Chron Respir Dis, 8, 253-8
crd.sagepub.com/content/8/4/253.abstract?etoc
• control of unnecessary coughing by education, breathing retraining and vocal hygiene

Patterson JE, Bradley JM, Hewitt O (2005) Airway clearance in bronchiectasis:
a randomized crossover trial of active cycle of breathing techniques versus Acapella. Respiration, 72, 239–242. DOI: 10.1159/000085363
• ACBT and the Acapella are equally effective, but patients often prefer the Acapella.

Phillips GE (1998) Airway response of children with primary ciliary dyskinesia in exercise and β2 agonist challenge. Eur Resp J, 11, 1389-91
• exercise is more beneficial than drugs in aiding bronchodilation before physiotherapy

Piciucchi S, Tomassetti S, Ravaglia C et al (2016) From “traction bronchiectasis” to honeycombing in idiopathic pulmonary fibrosis: A spectrum of bronchiolar remodeling also in radiology? BMC Pulm Med; 16:87. doi: 10.1186/s12890-016-0245-x
www.ncbi.nlm.nih.gov/pmc/articles/PMC4877976/

Pifferi M, Di Cicco M, Piras M et al (2013) Up to date on primary ciliary dyskinesia in children. Early Hum Dev; 89 Suppl 3:S45-8. doi: 10.1016/j.earlhumdev.2013.07.022

Quittner AL, O’Donnell AE, Salathe MA et al (2015) Quality of Life Questionnaire-Bronchiectasis: final psychometric analyses and determination of minimal important difference scores. Thorax;70:12-20. doi:10.1136/thoraxjnl-2014-205918
• validity, reliable and responsive patient-administered questionnaire.

Ramakrishnan VR, Ferril GR, Suh JD et al (2013) Upper and lower airways associations in patients with chronic rhinosinusitis and bronchiectasis. Int Forum Allergy Rhinol; 3(11):921-7. doi: 10.1002/alr.21204

Ramos EMC, Ramos D, Moreira GL et al (2015) Viscoelastic properties of bronchial mucus after respiratory physiotherapy in subjects with bronchiectasis. Respir Care; 60(5):724-730

Rea H, McAuley S, Donnell O et al (2004) Home based humidification therapy for patients with COPD and bronchiectasis. Eur Respir J, 24: Suppl. 48, 31s
www.ersnet.org/ers/lr/browse/media.aspx?id_dossier=14546&id_fiche=117613
• COPD & bronchiectasis pts find home humidification eases their cough and sputum production

Rea H, McAuley S, Jayaram L (2010) The clinical utility of long-term humidification therapy in chronic airway disease. Respir Med, 104, 525-33
• patients on long-term humidification had fewer exacerbations, higher QoL scores and improved lung function than those without.

Shapiro AJ, Zariwala MA, Ferkol T et al (2016) Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatric Pulmonol; 51(2):115-132. doi: 10.1002/ppul.23304.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4912005/

Soyza DA (2017) Bronchiectasis and aspergillus: how are they linked? Med Mycol; 1;55(1):69-81.

Stannard WA, Chilvers MA, Rutman AR et al (2010) Diagnostic testing of patients suspected of primary ciliary dyskinesia. Am J Respir Crit Care Med, 181, 4, 307-314. doi: 10.1164/rccm.200903-0459OC
• diagnosis of PCD by ciliary beat pattern analysis

Stillwell PC, Wartchow EP, Sagel SD et al (2011) Primary ciliary dyskinesia in children: a review for pediatricians, allergists, and pediatric pulmonologists. Pediatr Allergy Immunol Pulmonol, 24, 4, 191–196
www.ncbi.nlm.nih.gov/pmc/articles/PMC3255511/?tool=pmcentrez

Svenningsenab S (2017) Noncystic fibrosis bronchiectasis: regional abnormalities and response to airway clearance therapy using pulmonary functional magnetic resonance imaging. Academic Radiol; 24(1):4–12
• people with COPD have a 50% risk of bronchiectasis
• oscillatory PEP improves ventilation symptoms, exercise capacity and quality of life

Syed N (2009) Active Cycles of Breathing Technique (ACBT) versus conventional chest physical therapy on airway clearance in bronchiectasis – a crossover trial. Advances in Physiotherapy, 11, 4, 193-8
• both techniques equally effective, but patients tend to prefer ACBT

Takeuchi K, Kitano M, Ishinaga H et al (2016) Recent advances in primary ciliary dyskinesia. Auris Nasus Larynx; 43(3):229-36. doi: 10.1016/j.anl.2015.09.012

Tambascio J, de Souza HCD, Baddini M et al (2013) The influence of purulence on ciliary and cough transport in bronchiectasis. Respir Care; 58(12):2101-2106

Whitters D (2013) Bronchiectasis in older patients with Chronic Obstructive Pulmonary Disease. Drugs Aging; 30(4):215-25

Wickremasinghe S, Ozerovitch M, Davies G et al (2011) Non-tuberculous mycobacteria in patients with bronchiectasis. Thorax, 60, 12, 1045–1051
www.ncbi.nlm.nih.gov/pmc/articles/PMC3244167/?tool=pmcentrez


ACBT = active cycle of breathing
ACPRC = Association of Chartered Physiotherapists in Respiratory Care
BTS = British Thoracic Society
NIV = non-invasive ventilation
SGRQ = St George's Respiratory Questionnaire (scores symptoms, activity, social & psychological function)
SOB = shortness of breath