BRONCHIECTASIS

PRESENTER: [Link] V S

MODERATOR: DR. VIGNESSH RAVEE (AP)

Contents of slides

● Definition
● Etiology
● Theories of bronchiectasis
● Pathophysiology
● Classification
● Symptoms
● Signs
● Investigations
● Diagnosis
● Radiology imaging
● Complications
● Management
Definition

Irreversible, abnormal airway dilatation that involves the

lung in either a focal or a diffuse manner

● Destruction of smooth muscles and elastic tissue of

lung leading to irreversible dilatation of bronchi.
● Left lower lobe is the most common site
● Lower lobe>middle lobe>upper lobe
● Females>Males
● Diffuse: Alpha 1 antitrypsin deficiency, Conditions
Secondary to asthma ans constrictive bronchiectasis
● Upper lobe: Cystic Fibrosis, ABPA
● Lower lobe: immunodeficiency disease, primary ciliary
dyskinesia, Young’s syndrome, William Campbell and post
infectious aspiration
Etiology and pathogenesis

Pathogenic mechanisms Aetiology

Infection Bronchitis
● Pertussis
● Measles
● Adenovirus
Pneumonia
Tuberculosis

Impairment of mucous Cystic fibrosis

clearence Primary ciliary dyskinesia/Kartagener’s syndrome

Developmental pulmonary Pulmonary agenesis

anomalies (structural) Tracheobronchomegaly (Mounier-Kuhn
syndrome)
Bronchomalacia (Williams-Campbell syndrome)
Developmental defect Alpha1-antitrypsin deficiency
(Biochemical)

Infection secondary to Tumour

obstruction Aspirated foreign body
Lymphadenopathy

Immune response Allergic Broncho Pulmonary Aspergillosis

Auto immune Sjogren’s

Systemic lupus erythematous
Rheumatoid arthritis
Inflammatory bowel disease

Inhalational/aspiration injury Toxic fumes

Gastric contents
Theories of Bronchiectasis

● Atelectasis theory

● Pressure of secretion theory

● Traction theory
Atelectasis theory

● Aspiration of viscid material into peripheral parts of the

bronchial tree may result in atelectasis and dilatation of
the bronchi in the collapsed area

● Compensatory it reduces lung volume and increases

intrapulmonary negative pressure

● These in turn dilates any bronchi proximal to the block,

as these remain in communication with the atmosphere.
Pressure of secretion theory

• Secretions cause mechanical obstruction, which impairs mucus

clearance
• Results in accumulation of secretions distal to the obstruction
• Leading to secondary infections
• Thus causes inflammation, resulting in weakening and dilatation of
airway
Traction theory

● Bronchial dilatation occurs secondary to fibrosis

of lung parenchyma
● The resulting scar tissue requiring high inflation
pressures on inspiration to overcome
abnormally high retractive forces
Pathophysiology

Impaired mucus clearance

Vicious cycle Hypothesis for infectious

Pooling of secretion bronchiectasis

Bacterial proliferation

Recurrent infection

Neutrophil inflammation

Airway dilatation and damage

Mucociliary clearance

• Respiratory epithelium is lined with multiple motile cilia.

• These moves in a coordinated manner, forming an
escalator that clears overlying mucus from the airways .
• Mucus traps inhaled bacteria, viruses and particulate
matter, which are then transported by the cilia and
expectorated or swallowed.
• The mucociliary escalator is one of the first lines of
defense of the airways against infection
• Dysfunction of cilia and an increase of mucus leaves
the respiratory epithelium vulnerable to infection.
• patients with bronchiectasis show slow mucociliary
clearance and ciliary dyskinesia secondary to chronic
bacterial infection and neutrophilic inflammation.
• Bacterial infection and protease products (e.g.
neutrophil elastase) can also damage the ciliated
epithelium, negatively impacting on effective
clearance
 Airway infection
• Infection is the dominant stimulus for neutrophil recruitment to the airway
• Considered to be a major reason of disease progression in bronchiectasis
• Patients become chronically infected with Gram-negative organisms, such
as H. influenzae, P. aeruginosa, Moraxella catarrhalis and the
Enterobacteriaceae
• Gram-positive organisms, such as staph. aureus and streptococcus
pneumoniae.
• Gram-negative infection, more frequent as the disease becomes more
severe, suggesting a progressive impairment in antimicrobial defense.
Structural lung damage

• Structural abnormality of the bronchial wall in the form of

dilatation of the bronchi.
• The macroscopic pathology can range from cystic or
sacular bronchiectasis( large balloon-like terminal
dilatation of the bronchi, to less severe cylindrical
bronchiectasis) where there is often diffuse bilateral
dilatation
• Most commonly affecting the lower lobes.
● Mechanisms for non-infectious bronchiectasis

include immune-mediated reactions that damages the

bronchial wall (e.g., systemic autoimmune conditions such

as Sjögren’s syndrome, SLE and rheumatoid arthritis)

Mounier Kuhn syndrome

● Rare, congenital
● Cartilaginous rings of the trachea and its division till
segmental bronchi are enlarged, producing marked
dilatation of the trachea and central bronchi
● elastic and muscular tissues between the rings of cartilage
are atrophic and may bulge between the rings in the
manner of tracheal diverticulum.
● Associated with lower respiratory tract infection.
Williams-Campbell syndrome

● Also known as bronchomalacia

● Rare, congenital
● Characterized by defective or completely absent
bronchial wall cartilage, producing a mechanical
abnormality which leads to bronchiectasis.
● CT shows a remarkable ballooning expansion of the
proximal bronchi during inspiration, with collapse
during expiration
Kartagener’s syndrome

● Triad of chronic sinusitis,

bronchiectasis and situs inversus

● Are the results of impaired

mucociliary clearance from the

upper and lower respiratory tracts,

the paranasal sinuses and the

middle ear, male infertility

Allergic
●
bronchopulmonary aspergillosis
subjects, who also usually have asthma, are
affected by this condition which arises as a
result of allergy to Aspergillus spp.
● A. fumigatus being the fungus most
frequently implicated.
● These patients typically have ‘fleeting
infiltrates’ on their chest radiographs (or
rounded opacities in the case of ‘mucoid
impaction’)
● a positive immediate skin-prick test to A.
fumigatus, a peripheral blood eosinophilia
and a raised serum level of IgE,
● Aspergillus precipitins are detectable in
about 60% of typical cases.
Alpha 1- Antitrypsin deficiency

● Hereditary
● Homozygous alpha 1-antitrypsin deficiency (alpha1-
protease inhibitor)
● Associated with panacinar and basal emphysema, also
with cirrhosis of the liver
● Thus some of the proteases released from phagocytes
during pyogenic infection may be more liable to cause
bronchial wall damage if unopposed by antiproteases,
of which a1-antitrypsin is one.
Cystic fibrosis

● relatively common hereditary disorder

● in which activity of a chloride channel known as the cystic

fibrosis transmembrane conductance regulator (CFTR) is
reduced

● The diagnosis is made by finding the classic triad of

pulmonary disease, pancreatic insufficiency and a raised
sweat chloride concentration of 70mmol/L or more

● 90% of men with CF are azoospermic as a result of

congenital bilateral absence of the vas deferens
Infectious

● Pertussis (whooping cough)

Occurs in unvaccinated individuals
Produces a necrotizing bronchitis,and associated endo-bronchial
mucus and debris commonly cause small areas of atelectasis
● Measles
accompanied by severe inflammation of the bronchial wall and
pneumonia is its most important complication
● Adenovirus
account for about 5% of respiratory infections in children,
some serotypes (e.g. 1, 3, 4, 7 and 21)being more virulent.
Immunocompromised individuals-HIV
● human immunodeficiency virus infection is increasingly
recognized as a predisposing factor for repeated infection

● by other more common respiratory pathogens, such as

Streptococcus pneumoniae, Haemophilus influenzae,
Moraxella catarrhalis, Staphylococcus aureus and
Pseudomonas aeruginosa

● particularly in patients with less severe immunological

impairment such repeated infections may lead to
bronchiectasis
Common Varied Immunodeficiency (CVID)

● Also known as common variable hypogammaglobulinemia

● Presenting at any age, peak in childhood and adolescence
● Associated with chronic sinusitis, recurrent infective
bronchitis leading to bronchiectasis at adult life.
● Levels of immunoglobulins are variable
IgG : <2g/L
IgA : undetectable
IgM : <0.2g/L
● Functional immunoglobulin deficiencies should be
suspected in patients who have recurrent respiratory
infections with normal neutrophil and lymphocyte counts
Reid’s Classification
1. Cylindrical or tubular (most common)

Uniform dilatation of bronchi with straight lines

(regular outlines)

Tram track sign, signet ring sign in axial plane view

2. Varicose

Irregular dilatation of bronchi or beaded pattern of

bronchi resembling Varicose veins
3. Cystic/saccular

● Ballooning of bronchi

● Shows Tree In bud/ bunch of grapes/ honey

combing appearence

● Common in childhood

● Associated with Clubbing

Reid’s classification of Bronchiectasis

A) Cylindrical
B) Varicose
C) Cystic
Whitewells classification
• Follicular bronchiectasis
Presence of numerous lymphoid follicles, situated in thickened cylindrically
dilated bronchial walls

• Saccular bronchiectasis
Presence of macroscopically visible thin walled, saccular bronchial
dilatations

• Atelectatic bronchiectasis
Associated with pulmonary collapse, predominantly right sided
Clinical features

Symptoms
● Persistent cough with copius mucopurulent sputum
(most common)
three layered sputum top mucoid, middle
mucopurulent, and purulent layer at the bottom
● Hemoptysis (classical)
● Dyspnoea
● Wheeze if bronchial asthma associated
● General malaise, weight loss
● Pleuritic chest pain
Signs

● Persistent early mid inspiratory crackles

● Clubbing of the fingers/toes seen in prolonged bronchial

infection

● There may be Cyanosis, with signs of pulmonary

hypertension and right heart failure

Investigations
● Complete haemogram

● Serum total IgE and assessment of sensitization to [Link]

● Serum IgG, IgA & IgM (mainly IgA) in primary hypogammaglobulinemia

● Sweat electrolytes (cl) - Test for cystic fibrosis,

● Nasal clearance of saccharin, study of sperms-primary ciliary

dyskinesia

● Sputum culture

● 2D ECHO - Cor pulmonale

●
Imaging

● Plain Chest radiography is an unreliable means of

diagnosis

● High resolution CT is the investigation of choice

● Bronchography - rare
Diagnostic criteria in CT

● Airway diameter > accompanying blood vessel

● Broncho arterial ratio > 1 is pathological

● Lack of bronchial airway tapering

● Bronchial wall thickening in dilated airways, inspissated secretions

Radiological findings

● Typical Ring shadows produced by dilated bronchi

● Parallel lines ( tram track appearance)

● Gloved finger shadows (finger in glove appearance) due to

dilated bronchus containing mucous.

● Bunch of grape appearance

● Signet ring sign in HRCT

Typical Ring shadows

• Produced by dilated bronchi seen ‘end-

on
• May be small, numerous and
widespread, being one of the causes of
the radiographic appearance
sometimes referred to as honeycomb
lung.
• Larger (0.5–2cm diameter) ring
shadows may also occur in
bronchiectasis and sometimes contain
fluid levels, formed by retained
mucopus
Parallel lines

• Dilated bronchi viewed ‘side-on

• Fine parallel, air-containing, hair-line opacities may be seen in
normal subjects close to the hilum and represent a
longitudinal view of lobar or segmental bronchi.
• In bronchiectasis such opacities may be more obvious, their
walls being thickened rather than hair-line, and may be more
numerous or crowded together, indicating lobar shrinkage
• They are sometimes called ‘tramlines’
Tram track appearence
Signet ring sign
Bunch of grape appearence
Complications

● Massive hemoptysis

● Empyema with bronchopleural fistula

● Cor pulmonale/Right heart failure

● Lung abscess

● Secondary amyloidosis
Management

Medical treatment

● Chest Physiotherapy for removal of bronchial

secretion

● Postural drainage

● Antimicrobial therapy ( Macrolides and beta lactams)

● Bronchodilators
Antimicrobial therapy
● H. influenza and P. aeruginosa is the most common
Pathogen isolated in bronchiectasis
● strep. pneumoniae in exacerbation
● Macrolides and Beta lactams are the drug of choice
● Exacerbation : Oral/IV Antibiotics (macrolides-
azithromycin, clarithromycin) for 7-10 days,
● Frequent exacerbation with Pseudomonas infection:
Nebulised Polymyxin antibiotic colistin, tobramycin
● Mycobacterium avium complex (MAC) infection
Combination therapy with clarithromycin, rifampicin and
ethambutol
Surgical Treatment (children/young adults with localized
lesions who fail to respond to medical management,
recurrent hemoptysis, recurrent pneumonia)
● Surgical resection of bronchiectatic area-segment/lobe
● Lobectomy
● Lung transplant in advanced cases
Other supportive treatment
● Traditional mechanical chest percussion via hand clapping to the

chest

● Devices such as oscillatory positive pressure flutter valve

● High frequency chest wall oscillation vest

● Pulmonary rehabilitation

● Mucolytic dornase (DNase) only in Cystic Fibrosis related

bronchiectasis, reduces the viscosity of sputum.

 ● High-frequency chest wall
High frequency chest wall oscillation vest
oscillation involves an inflatable
vest that is attached to a machine.
● mechanically performs chest
physical therapy by vibrating at a
high frequency.
● The vest vibrates the chest to
loosen and thin mucus.
● Every five minutes, the person
stops the machine and coughs or
huffs.
Oscillatory positive pressure flutter valve

● Oscillating PEP (OscPEP) therapy provides

the combination of positive expiratory
pressure with high frequency oscillations.
● It involves breathing with a slightly active
expiration against an expiratory
resistance through a device.
● It is used for clearance of excess
secretions from the lungs
References

● Harrison’s principle of Internal Medicine 21st edition

● Crofton and Douglas’s Respiratory Diseases

● European Respiratory Society Monograph - Bronchiectasis