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Bronchiectasis

Authors: Heather I-Hsuan Chen-Yost, M.D., Kristine Konopka, M.D.

Editorial Board Member: Jefree J. Schulte, M.D.

Last author update: 11 October 2022

Last staff update: 20 January 2023

Copyright: 2003-2024, PathologyOutlines.com, Inc.

PubMed search: Bronchiectasis lung

Table of Contents

Cite this page: Chen-Yost HI, Konopka K. Bronchiectasis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumorbronchiectasis.html. Accessed December 24th, 2024.

Definition / general

Permanent dilation of bronchi and bronchioles caused by the destruction of mucosal and elastic tissues; often caused by or associated with chronic necrotizing infection of bronchi and bronchioles

Essential features

Gross pathology shows markedly distended peripheral bronchi, often with mucoid impaction and possible mucosal ulcerations
Histology shows a range of findings in the acute setting that include luminal dilation, mucostasis, acute inflammation and inflammatory debris
- Usual respiratory type epithelium may be ulcerated and replaced by granulation tissue or squamous metaplasia
- Chronic inflammation may be variably dense and include prominent lymphoid follicles and granulomas
- Chronic findings include loss of smooth muscle, cartilage and elastic fibers with replacement fibrosis
Can mimic squamous cell carcinoma on frozen section; on histology, need to distinguish from other lung diseases, such as chronic obstructive pulmonary disease (COPD), emphysema, cystic fibrosis and asthma

ICD coding

ICD-10:
- J47.9 - bronchiectasis, uncomplicated
- J47.0 - bronchiectasis with acute bronchitis or lower respiratory infection
- J47.1 - bronchiectasis with (acute) exacerbation
- Q33.4 - congenital bronchiectasis
ICD-11:
- CA24 - bronchiectasis
- 1B10.0 - respiratory tuberculosis, confirmed

Epidemiology

About 350,000 to 500,000 adults in the United States have bronchiectasis (Chron Respir Dis 2017:14:377)
Increased risk of noncystic fibrosis bronchiectasis with extremes of ages (< 5 years, adults > 75 years)
Associated with underserved populations
Increased incidence of childhood bronchiectasis in Maori, Pacific Islanders of New Zealand, Australian aboriginal and Alaskan native children (Expert Rev Respir Med 2017;11:517)

Sites

Bronchi walls in lungs

Pathophysiology

Obstruction (i.e., due to tumor, foreign body, inspissated mucus) (Int J Chron Obstruct Pulmon Dis 2009;4:411)
- Causes resorption of air distal to the obstruction
- Causes atelectasis and accumulation of intraluminal secretions
Nonobstructive bronchiectasis (i.e., due to pneumonia and atelectasis) (Int J Chron Obstruct Pulmon Dis 2009;4:411)
- Increased negative intrapleural pressure creates a force on bronchial walls, leading to dilation
Infectious bronchiectasis (N Engl J Med 2002;346:1383)
- Enhanced cellular and mediator responses lead to destruction of tissue
- Biopsies have shown infiltration by neutrophils and T lymphocytes
- Chemoattractants have been detected (IL8, TNFα, prostanoids)

Etiology

Idiopathic (Paediatr Respir Rev 2011;12:104)
Congenital
- Cystic fibrosis: most common cause in children
- Primary ciliary dyskinesia and other mucociliary disorders
- Immune deficiencies (hypogammaglobulinemia, MHC1 deficiency, TAP1 deficiency, IFNγ receptor deficiency)
- Alpha-1 antitrypsin deficiency
- Williams-Campbell syndrome
Infection
- Mycobacterial infection: most common cause globally
- Viral infection
- Allergic bronchopulmonary aspergillosis
- Postinfection sequelae (pneumonia, measles, whooping cough): most common, known noncystic fibrosis cause in adults
Autoimmune
- Rheumatoid arthritis
Other causes
- Chronic obstructive pulmonary disease / smoking
- Aspiration
- Extremes of age
- Immune dysfunction: HIV
- Foreign body / obstruction (i.e., tumor)
- Malnutrition

Diagrams / tables

Images hosted on other servers:

Morphological types

Different etiologies of bronchiectasis

Clinical features

Cough and chronic sputum production (Thorax 2019;74:1)
- Sputum is typically mucoid to mucopurulent, thick
- Can be bloody due to erosive airway damage
Dyspnea and wheezing
Pleuritic chest pain

Diagnosis

Physical exam (Thorax 2019;74:1)
- Abnormal breath sounds: crackles, wheezing, rhonchi
- Digital clubbing
CT scan to differentiate between bronchiectasis and COPD (can overlap)

Laboratory

CBC with differential
- Elevated blood platelets (> 400 x 10⁹/L in stable state) are associated with increased mortality (Ann Am Thorac Soc 2021;18:1316)
Depends on cause of bronchiectasis
- If infectious: sputum culture positive for infectious etiology
- If congenital: positive sweat chloride test, positive for genetic mutations, etc.
Immunoglobulin quantitation

Radiology description

Xray (Radiopaedia: Bronchiectasis [Accessed 25 August 2022]):
- Tram track opacities
- Air fluid levels
- Increase in bronchovascular markings
CT:
- Tram track sign: thickened nontapering bronchial walls
- Signet ring sign: dilated bronchus with pulmonary artery branch seen in cross section looks like a signet ring; bronchus and artery should be the same size
- String of pearls sign
- Bunch of grapes sign: closely apposed dilated bronchi appear to look like a bunch of grapes
- Bronchus visualized within 1 cm of pleural surface
- Lack of tapering
- Increased bronchoarterial ratio
- Bronchial wall thickening
- Mucoid impaction, air trapping

Radiology images

Images hosted on other servers:

CT, coronal cross section

CT, axial cross section

Prognostic factors

Bronchiectasis severity index
- Endorsed by the Thoracic Society of Australia and New Zealand
- Uses clinical, radiological and microbiological features to predict morbidity and mortality in noncystic fibrosis bronchiectasis
- Point system on a scale from 0 - 9
  - 0 - 4: mild bronchiectasis
  - 5 - 8: moderate bronchiectasis
  - 9+: severe bronchiectasis
- Calculator: University of Dundee: Bronchiectasis Prediction Tools [Accessed 25 August 2022]
FACED score (Open Respir Med J 2015;9:46)
- Uses a 0 - 7 point scale to determine the severity of bronchiectasis in noncystic fibrosis patients
- FACED stands for different metrics
  - F: forced expiratory volume in 1 second (FEV1) (≥ 50% = 0 points, < 50% = 2 points)
  - A: age (< 70 years = 0 points, ≥ 70 years = 2 points)
  - C: chronic colonization (no Pseudomonas = 0 points, presence of Pseudomonas = 1 point)
  - E: extension (1 - 2 lobes affected = 0 points, > 2 lobes affected= 1 point)
  - D: dyspnea; modified Medical Research Council scale (mMRC) (0 - 2 = 0 points, 3 - 4 = 1 point)

Case reports

42 year old man, a gardener with recurrent respiratory infections (Hosp Pract (Off Ed) 1985;20:97)
49 year old man who developed bronchiectasis after bronchial thermoplasty (J Thorac Dis 2018;10:E721)
52 year old man who developed COVID-19 associated bronchiectasis (Cureus 2021;13:e15051)

Treatment

Nonpharmacologic therapies (UpToDate: Bronchiectasis in Adults [Accessed 25 August 2022]):
- Avoid lung irritant
- Physiotherapy to clear airways
- Mucolytics
- Airway hydration
- Pulmonary rehabilitation for severe cases
Antibiotics to prevent exacerbations:
- Macrolides: especially for Pseudomonas aeruginosa
Other pharmacologic treatments:
- Bronchodilators
- NSAIDs
- Glucocorticoids

Gross description

Markedly distended peripheral bronchi, usually in lower lobes; can be traced up to pleura (Zander: Pulmonary Pathology, 2nd Edition, 2017)
- Variety of shapes present: saccular, cystic, cylindrical
Ulcerations of mucosal surfaces
Irregularly thickened bronchial walls
Honeycomb appearance on cut surface
Can have thick mucoid or mucopurulent secretions present in the bronchi

Gross images

Contributed by Kristine Konopka, M.D.

Dilated airways with mucoid deposition

Dilated airways

Frozen section description

Can be challenging on frozen section to distinguish from carcinoma (Int J Clin Exp Pathol 2015;8:7961)
- Bronchial epithelial shedding
- Epithelial squamous metaplasia: can be confused for carcinoma in situ
- Bronchial mural destruction

Frozen section images

Images hosted on other servers:

Squamous metaplasia in bronchiectasis

Microscopic (histologic) description

Inflammatory cells along airways (Zander: Pulmonary Pathology, 2nd Edition, 2017)
- Prominent chronic inflammation with lymphoid follicles and germinal centers
- Varying degrees of neutrophilic inflammation
- Can have eosinophils depending on the cause or if the patient has superimposed diseases
Epithelial ulceration
Over time
- Loss of smooth muscle, elastic fibers, cartilage
- Replacement fibrosis
Coexisting findings
- Organizing pneumonia
- Pulmonary hypertension

Microscopic (histologic) images

Contributed by Heather I-Hsuan Chen-Yost, M.D.

Lung explanted for cystic fibrosis

Diffuse inflammation and mucus plugging

Mixed acute and chronic inflammation

Lung explanted for idiopathic pulmonary fibrosis

Features of organizing pneumonia

Virtual slides

Images hosted on other servers:

Bronchiectasis with severe obstructive pneumonia

Mycetoma (aspergilloma) with background bronchiectasis

Cystic fibrosis (bronchiectasis with severe and chronic features)

Endobronchial hamartoma with postobstructive bronchiectasis

Cytology description

Seen in bronchial brushings and washings (Diagn Cytopathol 2008;36:13)
- Mixed inflammation
- Cellular debris
- Mucus
- Reactive bronchial cells
- Squamous metaplasia

Videos

Histopathology: lung bronchiectasis

Sample pathology report

Bilateral lungs, explant:
- Severe acute and chronic bronchiolitis with patchy fibrosis, consistent with bronchiectasis. Acute bronchopneumonia involving the left lower lobe.

Bilateral lungs, explant:
- Bronchiectasis with severe and necrotizing acute and chronic bronchitis and bronchiolitis consistent with cystic fibrosis.

Differential diagnosis

Many things can mimic or be superimposed with bronchiectasis but key findings can differentiate other diseases from it
- Bronchitis:
  - Increased goblet cells, thickened bronchial walls
- Asthma:
  - More eosinophils, goblet cell hyperplasia and metaplasia, Charcot-Leyden crystals, smooth muscle hypertrophy
- Aspiration pneumonia:
  - More neutrophils, abscesses, vegetable matter
- Tuberculosis:
  - Necrotizing granulomas
- Emphysema:
  - Gross findings with bullae
  - Alveolar wall destruction versus bronchiole destruction
- Lymphoid malignancies:
  - Monotonous lymphoid populations with abnormal germinal centers

Additional references

Am Rev Tuberc 1931;24:626, Curr Opin Infect Dis 2018;31:194

Board review style question #1

This histologic finding is most associated with which of the following congenital disease processes?

Carney complex
Congenital cystic airway malformation
Cystic fibrosis
Loeffler syndrome
Pulmonary lymphangiectasis

Board review style answer #1

C. Cystic fibrosis. The histologic finding seen here is bronchiectasis. While the other answers can be associated with bronchiectasis, the most common cause in this question would be cystic fibrosis. Loeffler syndrome is not a congenital disease (associated with Ascaris infection).

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Reference: Bronchiectasis

Board review style question #2

This explanted lung is most likely associated with which of the following diseases?

Asthma
Bronchiectasis
Emphysema
Interstitial fibrosis
Tuberculosis

Board review style answer #2

B. Bronchiectasis. The gross image of the explanted lung comes from a patient who had a lung transplant for cystic fibrosis disease. The bronchi walls are thickened, ulcerated, dilated and there is thick mucin present in the lumen. The dilation extends to the pleura. Emphysema would have more bullae. Asthma would also have mucin but also hyperinflation. Tuberculosis would most likely show granulomas, areas of caseating necrosis or miliary seeding. Interstitial fibrosis often shows honeycombing, prominent cobblestoning of the surface and may or may not have concurrent bronchiectasis.

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Reference: Bronchiectasis

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