BRONCHIECTASIS

by dr shams rehan

Definition
Bronchiectasis means
permanent dilatation of bronchi.
It can ensue whenever
bronchial obstruction is
accompanied by infection in the
collapsed portion of lung distal
to the obstruction

Classification

Congenital
Ciliary dysfunction syndrome
Ciliary dyskinesia syndrome or immotile cilia syndrome
Kartagener syndrome
Youngs syndrome
Alpha-1 antitrypsin deficiency
Cystic fibrosis
Pulmonary sequestration
Marfans syndrome
Immunodeficiency
Primary
Hypogammaglobulinemia
Secondary
Caused by cancer (CLL), chemotherapy, immune modulation
Acquired
Post-infectious conditions
Bacteria (pseudomonas, haemophilus)
Mycobacterium tuberculosis
Aspergillus species
Virus (adenovirus, measles, influenza, HIV)

Classification

Acquired (contd)

Diseases causing pulmonary fibrosis

CFA
Sarcoidosis
Allergic alveolitis
Bronchial adenoma or CA
Sequelae of toxic inhalation or aspiration
Chlorine
Overdose (heroin)
Foreign body
Rheumatic conditions
Rheumatoid arthritis
SLE
Sjgrens syndrome
Relapsing polychondritis
Other
Inflammatory bowel disease (chronic ulcerative colitis or crohns disease)
Yellow nail syndrome (yellow nails and lymphedema)

Types

Cylindrical or tubular bronchiectasis:

Uniformly dilated tubes that end abruptly at the
point that smaller bronchi are obstructed by
secretions

Varicose bronchiectasis:
The affected bronchi have irregular beaded
pattern of dilatation that resemble varicose veins

Saccular (cystic) Bronchiectasis:

The bronchi have a ballooned appearance at the
periphery, ending in blind sacs without
recognizable bronchial structures distal to that

Pathology

Clinical features

Symptoms

Asymptomatic
Cough
Sputum
production
Hemoptysis
Dyspnea
Malaise, fatigue
Fever
Wheezing

Signs

No signs
Digital clubbing
Fever
Coarse crepitations
Collapse
Fibrosis

Kartagener syndrome
Defects in ciliary ultrastructure & motility,
which ultimately impairs ciliary beating and
mucociliary clearance in upper & lower
respiratory tracts, paranasal sinuses & middle
ear.

Clinical presentation
Triad

Rhinosinus abnormalities
Situs inversus
Bronchiectasis

Infertilty

Youngs syndrome
Chronic sinusitis
Bronchitis
Bronchiectaiss
Obstructive azospermia

Cystic fibrosis
Hereditary disorder in which activity of chloride
channel known as cystic fibrosis transmembrane
conductance regulator (CFTR) is reduced

Pulmonary disease persistent lower resp tract

infection - Bronchiectasis

Pancreatic insufficiency
Raised sweat chloride concentration
Azoopsermia (90%) congenital bilat absence of vas
deferens

Comparison of Bronchiectasis & COPD

Variable

COPD

Bronchiectasis

Cause

Cig smoking

Infection, genetic or
immune defect

Role of infection

Secondary

Primary

Predominant
organism in sputum

Strep pneumoniae, H influenzae

H influenzae, Ps aeroginosa

Airflow obstruction &

hyperresponsiveness

Present

Present

Finding on CXR

Hyperlucency, hyperinflation,
airway dilatation

Airway dilatation &

thickening, mucus plugs

Quality of sputum

Mucoid, clear

Purulent, three layered

Investigations

Sputum examination (microscopy, culture & AFBs)

Chest X Ray
Sinus radiographs
HRCT Scan chest
Spirometry
Bronchography (Historical)
Fiberoptic bronchoscopy
Specialized tests
Mucociliary function - ciliary beat frequency
Imunoglobulin levels
Sweat chloride test (>80mmol/l)
Semen analysis
Tests for aspergillus
Antibody testing
Antigen detection in serum & urine

Investigations

Chest Xray

Focal pneumonitis
Scattered irregular opacities
Pulmonary collapse & fibrosis
Linear or plate like atelectasis
Dilated & thickened airways
Ring shadows
Tram-lines
Gloved finger shadow
Pulmonary hypertension
Situs inversus

Chest X Ray

HRCT Scan
Markedly dilated airways

Ring shadows
partially filled with
secretions

HRCT Scan
Severe cystic
bronchiectasis,
cicatrization
atelectasis

Dilated thick airways

(signet rings)

Spirometry
Airflow

limitation (reduced FEV1/FVC)

Normal or slightly reduced FVC
Reduced FEV1

Complications
Infection within dilated bronchi
Infection spread to lungs and pleura
Distant spread of infection
General effects of chronic infection within bronchi

Amyloid disease
Pneumothorax
Cor pulmonale
Respiratory failure
Secondary infection
Invasive broncho pulmonary aspergillosis

Causes of death from

bronchiectasis

Massive hemoptysis
Cor pulmonale
Respiratory failure
Secondary infection leading to
pneumonia and septicemia
Invasive bronchopulmonary
aspergillosis

Treatment

Postural drainage
Antibiotics

Amoxicillin, Augmentin
Azithromycin, Clarithromycin
Antipseudomonal agent (Pseudomonas aeroginosa)

Mucolytic agents
Recombinant human DNAse (dornase alfa)
Corticosteroids for airway obstruction, ABPA
Prednisolone 30-40mg/day

GERD
Immunoglobulins- for immunodeficiency
syndromes
Surgery
Palliative surgical resection - Lobectomy,
pneumonectomy
Heart lung transplant