Bronchiectasis
Lecture by DR AISHA IMRAN
Bronchiectasis
Definition:
Bronchiectasis is a chronic lung
disease characterized by
permanent and irreversible dilation
of the bronchi and bronchioles due
to destruction of the elastic and
muscular components of the
bronchial walls. It leads to chronic
infection, inflammation, and
impaired mucociliary clearance.
Etiology (Causes)
Bronchiectasis can be congenital
or acquired.
Congenital Causes
Cystic Fibrosis (most common
genetic cause)
Primary Ciliary Dyskinesia
(Kartagener Syndrome)
Alpha-1 Antitrypsin Deficiency
Acquired Causes
Post-infectious (Most common):
Tuberculosis
Pneumonia
Measles,
Pertussis
Obstruction:
Foreign body inhalation
Tumors
Autoimmune Diseases:
Rheumatoid arthritis
Inflammatory Bowel Disease (IBD)
Aspiration (due to GERD,
neuromuscular disorders)
Immunodeficiency (e.g., HIV,)
Pathophysiology
Infection or Obstruction →
Damage to bronchial walls
Inflammation & Weakening →
Destruction of cilia, mucus
accumulation
Airway Dilation & Distortion →
Permanent bronchial damage
Chronic Cycle → Recurrent
infections → More inflammation →
Clinical Features
Chronic cough (productive,
purulent sputum)
Recurrent lung infections
Hemoptysis (blood-streaked
sputum)
Dyspnea (shortness of breath)
Wheezing & Crackles (on
auscultation)
Digital Clubbing (in severe cases)
Diagnosis
High-Resolution CT (HRCT) Scan
(Gold standard)
Signet ring sign (dilated bronchi
with adjacent pulmonary artery)
Tram-track sign (parallel
thickened bronchi)
Chest X-ray
Dilated bronchi, cystic spaces
Signet ring sign
Tram-track sign
Sputum Culture
Common pathogens:
Pseudomonas aeruginosa,
Staphylococcus aureus,
Haemophilus influenzae
Pulmonary Function Test (PFTs)
Obstructive pattern (↓ FEV1/FVC
ratio)
Bronchoscopy (for identifying
obstruction)
Management
1.Airway Clearance Therapy
Postural drainage & Chest
physiotherapy
High-frequency chest wall
oscillation (vest therapy)
Nebulized hypertonic saline
(mucus clearance)
Antibiotics(For acute
exacerbations & prophylaxis in
severe cases)
Empirical: Amoxicillin-
clavulanate, Macrolides
(Azithromycin)
Pseudomonas infection:
Ciprofloxacin, Inhaled Tobramycin
MRSA: Vancomycin or Linezolid
Bronchodilators (For airflow
obstruction)
Short-acting beta-agonists
(SABAs): Salbutamol
Long-acting beta-agonists
(LABAs) with inhaled
corticosteroids
Mucolytics & Hydration
N-acetylcysteine (Mucolytic)
Adequate fluid intake
Surgery (For localized disease &
complications)
Lobectomy (if localized and
symptomatic)
Lung transplantation (end-stage
disease)
Complications
Recurrent lung infections →
Respiratory failure
Massive hemoptysis
Pulmonary hypertension → Right
heart failure (Cor Pulmonale)
Abscess formation
Physiotherapy Management
Postural Drainage & Percussion
(gravity-assisted drainage)
Breathing Exercises (Pursed-lip
breathing)
Physical Activity & Pulmonary
Rehabilitation
Cystic Fibrosis (CF)
Definition
Cystic fibrosis (CF) is a genetic,
autosomal recessive disorder affecting
the CFTR (Cystic Fibrosis Transmembrane
Conductance Regulator) gene, leading to
defective chloride ion transport. This
results in thick, sticky mucus in the
lungs, pancreas, intestines, and other
organs, causing chronic respiratory
infections, pancreatic insufficiency, and
malabsorption.
Etiology (Causes)
Genetic Mutation: Mutations in
the CFTR gene (Chromosome 7)
Most Common Mutation: ΔF508
(Delta F508) – causes misfolding
of CFTR protein, leading to its
degradation before reaching the
cell membrane.
Pathophysiology
Defective CFTR protein → Decreased
chloride secretion → Increased sodium
and water absorption.
Thickened mucus secretions → Clogs
airways and ducts.
Lung infections & inflammation →
Chronic lung disease.
Pancreatic insufficiency → Malabsorption,
fat-soluble vitamin deficiency.
Meconium ileus (newborns) → Intestinal
obstruction.
Clinical Features
Respiratory System
Chronic cough (productive, thick
sputum)
Recurrent lung infections (Pseudomonas
aeruginosa, Staphylococcus aureus)
Bronchiectasis (permanent airway
dilation)
Nasal polyps, Sinusitis
Digital clubbing (late-stage CF)
Gastrointestinal System
Pancreatic insufficiency → Fatty
stools (steatorrhea), malnutrition
Meconium ileus (intestinal
obstruction in newborns)
Fat-soluble vitamin deficiencies
(A, D, E, K)
Cystic fibrosis-related diabetes
(CFRD)
Reproductive System
Male infertility (Congenital
absence of the vas deferens)
Delayed puberty in both sexes
Sweat Glands
Salty skin (High chloride levels in
sweat)
Diagnosis
1. Newborn Screening
Immunoreactive Trypsinogen (IRT)
(Elevated in CF)
2. Sweat Chloride Test (Gold Standard)
Chloride > 60 mmol/L confirms CF
3. Genetic Testing
Identifies CFTR mutations (ΔF508 most
common)
4. Pulmonary Function Tests
(PFTs)Obstructive pattern (↓ FEV1/FVC
ratio)
Management
1. Airway Clearance
Chest Physiotherapy (Postural
drainage, percussion)
Nebulized Hypertonic Saline
(Mucus clearance)
DNase (Dornase Alfa)
(Mucolytic, breaks down mucus
DNA)
2. Antibiotic Therapy (For
Recurrent Infections)
Pseudomonas aeruginosa:
Inhaled Tobramycin, Aztreonam
MRSA: Vancomycin, Linezolid
3.Bronchodilators& Anti-
Inflammatory Therapy
Salbutamol (β2 agonist) – Opens
airways
Azithromycin (Macrolide) – Anti-
inflammatory & antimicrobial
4. Pancreatic Enzyme
Replacement Therapy
(PERT)Pancrelipase (Lipase,
Amylase, Protease) for digestion
5. Nutritional SupportHigh-
calorie, high-fat diet
Fat-soluble vitamin supplements
(A, D, E, K)
6. CFTR Modulators (Targeting
Genetic Defect)
Ivacaftor (For G551D mutation) –
Improves CFTR function
Lumacaftor + Ivacaftor (For
ΔF508 mutation) – Increases
CFTR protein at the membrane
7. Lung Transplant (For End-stage
CF)
Complications
Respiratory Failure (leading cause
of death)
Pulmonary Hypertension → Cor
Pulmonale
Cystic Fibrosis-Related Diabetes
(CFRD)
Osteoporosis (Vitamin D
deficiency)
