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C. J. Rees et al.
• CT ndings in bronchiectasis include airway dilatation, lack of bronchial
tapering (usually noticed as tubular structures within 1cm of the pleural surface), bronchial wall thickening in dilated airways, and a large amount of
dried secretions (tree-in-bud pattern).
• The signet-ring sign on CT is when there is a cross-sectional airway with a
diameter that is at least 1.5 times larger than an adjacent vessel.
(CT Thorax): Resolution of areas of consolidation and “signet ring sign”. The signet
ring sign is a nding seen on CT scans of the thorax. It consists of a small circle of
soft tissue attenuation that abuts a ring of soft tissue attenuation surrounding a larger
low attenuating circle of air and is indicative of bronchiectasis. [From article:
Invasive pulmonary aspergillosis 10 years post bone marrow transplantation: a case
report. Journal of Medical Case Reports. 2009;3(1):26. https://doi.org/10.1186/1752-
1947-3-26, at http://link.springer.com/article/10.1186%2F1752-1947-3-26/fulltext.
html; by Rifat Rashid, David W Denning, © Rashid and Denning; licensee BioMed
Central Ltd. 2009; licensed under Creative Commons Attribution License BY 2.0
http://creativecommons.org/licenses/by/2.0] Caption adapted from original.
14 Bronchiectasis
241
High resolution chest computed tomography scan (lung window) showing (a)
mucus-lled dilated bronchi (arrow) and extensive bilateral central bronchiectasis
characterised by (b) “string of pearls” and (c) “signet ring” appearances [Shah A.
How to Diagnose Allergic Bronchopulmonary Aspergillosis. In: Comarú Pasqualotto
A, editor. Aspergillosis: From Diagnosis to Prevention [Internet]. Dordrecht: Springer
Netherlands; 2009 [cited 2015 Nov 19]. p.725–45. Available from:
http://www.spring-
erlink.com/index/10.1007/978-90-481-2408-4_43] Caption from original.
Computed tomographic image of bronchiectasis. Chest CT showing diffuse dilated
bronchi (bronchiectasis), especially in the right lower lobe. Thick-walled bronchi
are seen. Also present is the “signet-ring” sign (arrow), in which the diameter of the
airway (lumen) is greater than that of the adjacent vessel. Apparent in the right
middle lobe is “tram-tracking” (arrowhead), with absence of tapering of the peripheral bronchi. [MacLusky I, Solomon M, Laxer R, Ford-Jones EL, Friedman J,
Gerstle T. Atlas of Pediatrics, Volume IA, Chapter 14. In: Laxer RM, editor. The
Hospital for Sick Children: Atlas of Pediatrics. Philadelphia, PA: Current Medicine
Group; 2005. 519 p. ISBN 1-57340-188-9] Caption from original.
242
• Most patients with bronchiectasis will undergo bronchoscopy during evaluation. This is most important in patients with focal bronchiectasis to exclude
airway obstruction from a mass or foreign body.
• Pulmonary function testing can help in gauging the functional impairment of
the patient.
C. J. Rees et al.
Special Populations
Age
• The incidence of bronchiectasis increases with age.
• Patients with cystic brosis can develop clinically signicant bronchiectasis
in adolescence or young adulthood.
Co-morbidities
• Co-morbidities of interest include all the etiologies listed above.
• Bronchiectasis may coexist with COPD and other chronic lung diseases.
• Bronchiectasis may also coexist with causes of chronic dyspnea and cough,
such as congestive heart failure.
Pitfalls inDiagnosis
Critical Steps Not toMiss
• Consideration of the diagnosis is the rst critical step.
• It is important to perform appropriate imaging in patients in whom the diagnosis is being considered.
• A search for the possible etiology should be performed for all patients being
evaluated for bronchiectasis.
Mimics
• Typical mimics of bronchiectasis include COPD/emphysema, asthma, and
pulmonary brosis.
14 Bronchiectasis
Time-Dependent Interventions
• Time-dependent interventions in bronchiectasis are rarely necessary. This is a
chronic, slowly progressive disease.
• Acute exacerbations often benet from the timely use of antibiotics, as well
as from bronchial hygiene measures (help to enhance secretion clearance)
such as chest physiotherapy, mucolytics, hydration, and sometimes bronchodilator therapy.
Overall Principles ofTreatment
• The general treatment of bronchiectasis involves:
• control of any active infection.
• attempts to improve bronchial hygiene to help minimize the risk of repeated
infections.
• Antibiotics are usually administered for acute exacerbations. These are
suspected clinically in the presence of a worsened cough, qualitative (and
often quantitative) change in the sputum, and sometimes fever. The most commonly isolated causative organisms of acute infectious exacerbations are
Haemo philus inuenza, and Pseudomonas aeruginosa. The typical antibiotics
used are uoroquinolones, such as ciprooxacin and levooxacin.
• The decision to treat Mycobacterium avium-intracellulare complex (MAC) is
difcult. This organism can exist as a colonizer as well as a pathogen, and the
treatment regimens are prolonged and often poorly tolerated. MAC, however,
is the most common non-tuberculous mycobacterial cause of bronchiectasis.
The diagnosis of this infection, and the decision to treat, is often guided by
expert opinion.
• Multiple approaches are usually taken to try and enhance secretion clearance
(bronchial hygiene) in these patients.
243
• Maintaining adequate hydration and the use of mucolytics to keep
secretions thin.
• Use of bronchodilators.
• Chest physiotherapy, including chest percussion and newer mechanical
devices.
• The use of systemic glucocorticoids is controversial. There has been no
compelling evidence demonstrating improvements in pulmonary function or
of lower exacerbation rates from their use.
• Oral or systemic glucocorticoids may be necessary for some patients
depending upon the etiology of their bronchiectasis. Patients with allergic
bronchopulmonary aspergillosis, Rheumatoid arthritis, and Sjogren’s
244
syndrome may benet from glucocorticoids for both their bronchiectasis
and their underlying disease.
• In severe or refractory cases, surgery (such as resection of involved lung
tissue) may be considered. This is especially true of patients with focal
bronchiectasis.
• End-stage patients may be considered for lung transplantation.
• All patients with chronic respiratory conditions should be kept up to date on
vaccinations, especially inuenza and pneumococcal. Appropriate use of
these vaccinations can help reduce the rate of recurrent infections.
• Patients who smoke should be counseled about cessation and offered help
with quitting.
C. J. Rees et al.
Disease Course
• Bronchiectasis is a chronic, recurrent, progressive disease.
• Outcomes and course vary widely based upon the underlying etiology.
• The decline in lung function is similar to that seen in patients with COPD.
Related Evidence
Papers of particular interest have been highlighted as:
** Of key importance
Practice Guideline
Pasteur MC, Bilton D, Hill AT; British Thoracic Society Bronchiectasis non-CF
Guideline Group. British Thoracic Society guideline for non-CF bronchiectasis.
Thorax. 2010 Jul;65 Suppl 1:i1-58. https://doi.org/10.1136/thx.2010.136119.
PMID: 20627931. http://www.ncbi.nlm.nih.gov/pubmed/20627931 **
Review
Sidhu MK, Mandal P, Hill AT.Bronchiectasis: an update on current pharmacother-
apy and future perspectives. Expert Opin Pharmacother. 2014 Mar;15(4):505-25.
https://doi.org/10.1517/14656566.2014.878330. PMID: 24410485. http://www.
ncbi.nlm.nih.gov/pubmed/24410485 **
14 Bronchiectasis
245
Lee AL, Burge A, Holland AE.Airway clearance techniques for bronchiectasis.
Cochrane Database Syst Rev. 2013 May 31;5:CD008351. https://doi.
org/10.1002/14651858.CD008351.pub2. PMID: 23728674. http://www.ncbi.
nlm.nih.gov/pubmed/23728674
**
McShane PJ, Naureckas ET, Tino G, Strek ME. Non-cystic brosis bronchiec-
tasis. Am J Respir Crit Care Med. 2013 Sep 15;188(6):647-56. https://doi.
org/10.1164/rccm.201303-0411CI. PMID: 23898922. http://www.ncbi.nlm.
nih.gov/pubmed/23898922 **
McDonnell MJ, Ward C, Lordan JL, Rutherford RM.Non-cystic brosis bronchiec-
tasis. QJM. 2013 Aug;106(8):709-15. https://doi.org/10.1093/qjmed/hct109.
PMID: 23728208. http://www.ncbi.nlm.nih.gov/pubmed/23728208 **
Bonavita J, Naidich DP. Imaging of bronchiectasis. Clin Chest Med. 2012
Jun;33(2):233-48.
https://doi.org/10.1016/j.ccm.2012.02.007. PMID: 22640843.
http://www.ncbi.nlm.nih.gov/pubmed/22640843 **
Moulton BC, Barker AF. Pathogenesis of bronchiectasis. Clin Chest Med. 2012
Jun;33(2):211-7.
https://doi.org/10.1016/j.ccm.2012.02.004. PMID: 22640841.
http://www.ncbi.nlm.nih.gov/pubmed/22640841 **
Singer LG, Herridge MS.Clinical year in review II: bronchiectasis, mycobacterial
infections of the lung, sleep-disordered breathing, and lung transplantation. Proc
Am Thorac Soc. 2010 Sep;7(5):305-11. https://doi.org/10.1513/pats.201007-
051TT. PMID: 20844288. http://www.ncbi.nlm.nih.gov/pubmed/20844288 **
Goeminne P, Dupont L.Non-cystic brosis bronchiectasis: diagnosis and manage-
ment in 21st century. Postgrad Med J. 2010 Aug;86(1018):493-501. https://doi.
org/10.1136/pgmj.2009.091041. PMID: 20709772. http://www.ncbi.nlm.nih.
gov/pubmed/20709772 **
Cantin L, Bankier AA, Eisenberg RL.Bronchiectasis. AJR Am J Roentgenol. 2009
Sep;193(3):W158-71. https://doi.org/10.2214/AJR.09.3053. PMID: 19696251.
http://www.ncbi.nlm.nih.gov/pubmed/19696251 **
Use PubMed Clinical Queries to nd the most recent evidence. Use this search
strategy:
“Bronchiectasis”[Mesh] OR “Bronchiectasis”
Chapter 15
Bronchiolitis
RichardM.Cantor, CharlesV.Pollack,Jr., andVictoriaG.Riese
Name andSynonyms
Formally known as Respiratory Syncytial Viral (RSV) pneumonia, but many other
viruses (such as metapneumovirus, adenovirus, parainuenza virus) may cause the
same clinical disease.
Incidence/Epidemiology
• Typically affects infants under 2 years of age.
• Reported peak incidence between 2 and 6 months.
• Seasonal peaks during fall and winter.
• Bronchiolitis is the leading cause of hospitalization in young children.
R. M. Cantor
Department of Emergency Medicine and Pediatrics, State University of NewYork Upstate
Medical University, Syracuse, NY, USA
C. V. Pollack,
Department of Emergency Medicine, Thomas Jefferson University,
Philadelphia, PA, USA
V. G. Riese
Librarian Consultant, Eldersburg, MD, USA
C. V. Pollack, Jr. (ed.), Differential Diagnosis of Cardiopulmonary Disease,
https://doi.org/10.1007/978-3-319-63895-9_15
Jr. ()
247© Springer Nature Switzerland AG 2019
248
R. M. Cantor et al.
Incidence rates of hospitalizations for bronchiolitis by age (in months) (section A)
and by calendar month (section B) in pre-term infants; Rome, Italy 2000-2006.
[From article: Incidence and risk factors of hospitalization for bronchiolitis in preterm children: a retrospective longitudinal study in Italy. BMC Pediatrics. 2009 Sept
10; 9:56; https://doi.org/10.1186/1471-2431-9-56, at http://bmcpediatr.biomedcen-
tral.com/articles/10.1186/1471-2431-9-56; by Patrizio Pezzotti, Jessica Mantovani,
Nicoletta Benincori, Eleonora Mucchino, Domenico Di Lallo, © Pezzotti et al;
licensee BioMed Central Ltd. 2009; licensed under Creative Commons Attribution
License BY 2.0 http://creativecommons.org/licenses/by/2.0] Caption from original
Differential Diagnosis
The typical affected infant presents with fever and wheezing, and as such initially
prompts these differential considerations:
• Bacterial pneumonia
• Chlamydial pneumonia
• Foreign-body aspiration
• Reactive airway disease/asthma
• Aspiration pneumonia
• Congenital heart disease
• Vascular rings
15 Bronchiolitis
249
The problem representation allows identication of three illness scripts that t the
dening features of this toddler with respiratory distress case. Diffuse lower respiratory
ndings on auscultation is the key discriminating feature, which allows a diagnosis of
bronchiolitis [Mutnick A, Barone M.Assessing and Remediating Clinical Reasoning.
In: Kalet A, Chou CL, editors. Remediation in Medical Education [Internet]. NewYork,
NY: Springer NewYork; 2014 [cited 2016 Jul 28]. p.85–101. Available from: http://
link.springer.com/10.1007/978-1-4614-9025-8_6] Caption from original
Pathophysiology andEtiology
• Viral inltration of terminal bronchioles results in edema, increased mucous
production, and sloughing of respiratory epithelial cells.
250
Inuenza virus infection. Like other forms of respiratory viral infection, inuenza may cause a lymphocytic or necrotizing bronchiolitis, in this case associated with mucus stasis in the bronchioles (H&E, 40×). Autopsy examination
following fatal inuenza viral infection typically shows a necrotizing bronchitis
and bronchiolitis with pulmonary edema and diffuse alveolar damage in the
background (not shown) [Shah KK, Dishop MK. Infantile Viral Illnesses. In:
Fraire AE, Woda BA, Welsh RM, Kradin RL, editors. Viruses and the Lung
[Internet]. Berlin, Heidelberg: Springer Berlin Heidelberg; 2014 [cited 2016 Jul
28]. p. 143–58. Available from:
40605-8_17] Caption from original
• Lymphocytic inltration causes the typical pattern of peribronchial cufng
seen on plain chest radiographs.
• Known causative viruses include RSV, metapneumovirus, rhinovirus, parainuenza virus, and adenovirus.
http://link.springer.com/10.1007/978-3-642-
R. M. Cantor et al.
Presentation
Typical/“Classic”
• There is often a 2 to 4-day prodrome consisting of cough and rhinorrhea.
• Infants present after the prodrome with fever, increasing cough, and variable
degrees of respiratory distress.
• Patients manifest a wide range of work of breathing, characterized by tachypnea, expiratory prolongation, retractions, and in more severe cases,
grunting.
Atypical
• In infants less than 6 weeks of age, apneic episodes may be the rst sign of
bronchiolitis.
Primary Differential Considerations
• Early diagnostic consideration should also be given to:
• Asthma
• Pneumonia
• Croup