Bronchiectasis Flashcards
What are the symptoms of bronchiectasis?
1- Cough: usually purulent, daily and persistent
2-Sputum production: large amounts and purulent.
3-Breathlessness( in advanced patients) : as disease progresses.
4- Hemoptysis( life threatening) : usually a sign of infection; streaking is common but massive hemoptysis is a rare medical emergency.
5- Infection: usually characterized by increased sputum volume and increased
purulence (infective exacerbation) .
6-Pleuritic chest pain : can be a feature of infection, as well as fever.
7-Coarse crackles: heard on auscultation but examination can be normal or
inspiratory “squeaks” .
8- Clubbing: especially in cystic fibrosis.
The last two are very important.
What is bronchiectasis?
Bronchiectasis describes irreversible abnormal permanent dilatation of the airways.
Chronic suppurative airway infection with sputum production, progressive scarring and
lung damage occur, whatever the cause.
Wha is Bronchiectasis Pathology?
• It is an obstructive airways disease of bronchi and bronchioles, chronic
transmural inflammation with airway dilatation and thickening, collapsibility,
mucus plugging with impaired clearance.
• The disease is characterized by a vicious circle of neutrophilic inflammation,
recurrent infection and damage to the airway. This further impairs mucociliary
clearance, and persistent inflammation leads to impairment of immunity.
What is the etiology of Bronchiectasis?
• Globally, Tuberculosis (TB) is the leading cause of bronchiectasis.
• Bronchiectasis associated with other lung diseases in particular, COPD – is becoming
increasingly recognized.
What are the Causes of Bronchiectasis?
1- Mucociliary clearance defects:
A) Primary ciliary dyskinesia (Kartagener’s syndrome: with dextrocardia and
situs inversus, chronic sinusitis) and
B)Cystic fibrosis.
2-Mechanical bronchial obstruction:
A) Intrinsic: Foreign body, mucus plug, Post-tuberculous and stenosis and Tumor.
B)Extrinsic: Lymph node, Tumor
3- Post infective bronchial damage:
• Bacterial and viral pneumonia, including pertussis, measles and aspiration pneumonia.
4- Autoimmune diseases:
• Rheumatoid arthritis (RA), Systemic lupus erythematous (SLE) .
5- Inflammatory bowel diseases:
• Crohn’s disease and ulcerative colitis
6-Granuloma:
• Tuberculosis, sarcoidosis
7-Diffuse diseases of the lung parenchyma:
• e.g. Idiopathic pulmonary fibrosis.
8-Immunological over-response:
• Allergic bronchopulmonary aspergillosis
• Post-lung Transplant
9-Immune deficiency:
• Primary: Panhypogammaglobulinaemia, Selective immunoglobulin deficiencies (IgA and
IgG2)
• Secondary: Human immunodeficiency virus and malignancy.
10- α1-Anti-Trypsin deficiency?
What are the diagnostic studies Bronchiectasis?
1- Sputum examination
- is useful for a focused antibiotic treatment plan, as well as the exclusion of non-
tuberculous mycobacterial disease .
In addition to common respiratory pathogens , sputum culture may reveal
Pseudomonas aeruginosa and Staphylococcus aureus, fungi such as Aspergillus and
various mycobacteria.
2-Chest X-ray
- May often be normal but in advanced patients tram track airways, ring shadows and cysts may be seen.
3-HRCT (high resolution CT) scanning:
is the investigation of choice (gold standard).
Characteristically, non-tapering
A)-‘tram track’ airways,
B)- bronchial wall thickening
C)- and an increased bronchoarterial ratio termed the ‘signet ring’ sign can be seen.
4- PFT: obstructive pattern.
5- Immune assessment:
would include immunoglobulins and responses to Hemophilus influenzas, tetanus
and pneumococcal vaccines as baseline tests.
6-Nasal nitric oxide:
• is a useful test for screening for primary ciliary dyskinesia (PCD). It is very low in
PCD.
What can we see in HRCT in Bronchiectasis patients?
1- tram track.
2- bronchial wall thickening.
3- signet ring.
How to manage patients with Bronchiectasis?
Therapy can broadly be divided into
-Vaccination (influanza and pneumococcal).
• Airway clearance
• Anti-inflammatories
• Treatment of infection and complications by using antibiotics.
1- Treat underlying cause:
• Acute exacerbations: antibiotics directed against prior pathogens; if no prior
culture data → fluroquinolone. (FLQ)
2- Airway clearance :
A) Daily airway clearance therapies are advised. (self-) drainage and postural drainage
are popular modalities.
B) chest physiology.
C) Nebulized hypertonic saline is also approved for use in bronchiectasis; it works as a
mucoactive agent.
3- Anti-inflammatories:
• Long-term azithromycin has an immunomodulatory effect and has been
demonstrated to reduce exacerbation frequency . (shown to ↓ exacerbation in non-
CF bronchiectasis)
• Inhaled/oral corticosteroids are beneficial to some patients.
4- treatment of infection:
Treatment of exacerbations usually lasts 2 weeks and is based on previously obtained
microbiological information. if no prior culture data → Fluoroquinolones
*** When Pseudomonas aeruginosa is being treated, dual therapy is often used where there
are multi-resistant pathogens and where multiple antibiotic courses would be expected.
————->High-dose ciprofloxacin (750 mg twice daily) is a useful oral drug for treatment of
Pseudomonas.
-H. influenzae infection is common in bronchiectasis and usually responds to oral
antibiotics such as amoxicillin, co-amoxiclav or doxycycline.
5-Pulmonary rehabilitation should be offered to patients with a reduce exercise capacity
and breathlessness.
6-Massive haemoptysis is a life-threatening medical emergency treatment is resuscitation
with airway protection until bronchial artery embolization can be performed to control
the bleeding.
7- Respiratory failure should be treated with oxygen and non-invasive ventilation .
Suitable patients should be referred to a transplant center.
What are the prognosis of Bronchiectasis?
Prognosis is undefined and obviously quite variable, depending on disease severity.
• A low FEV1 and infection with P. aeruginosa are associated with a poor outcome.
What is cystic fibrosis?
.CF is an autosomal recessive condition due to mutations in chloride channel (CFTR gene) ( chromosome 7).
The F508del mutation is the most common, accounting for around 70% of cases.
survival has improved dramatically.
• The current expected median survival is now around 47 years.
What is Pathogenesis of Cystic Fibrosis?
CF is a multisystem disease, although respiratory problems are usually the most
prominent. A vicious circle of mucus stasis, inflammation and infection leads to
respiratory failure and death in the majority of patients.
In the lungs, CFTR dysfunction leads to dehydrated airway surface liquid, mucus
stasis, airway inflammation and recurrent infection.
↑ mucus thickness, ↓ mucociliary clearance, ↑ infections → Bronchiectasis
What are Clinical presentations and complications of CF in respiratory ?
Respiratory:
• Recurrent respiratory infection.
• Chronic daily cough and sputum production.
• Breathlessness.
• Nasal polyps.
• Hemoptysis (sometimes massive)
• Pneumothorax
• Respiratory failure and cor pulmonale
• Recurrent sinusitis
What are the Clinical presentations and complications of CF in GI?
*Failure to thrive in infancy and low body mass index in adults.
• Meconium ileus in infancy
• Distal intestinal obstruction syndrome.
• Steatorrhea secondary to pancreatic insufficiency .
• Cystic fibrosis-related liver disease and cirrhosis
• Increased risk of gastrointestinal malignancy.
What are the complication of CF?
*Cystic fibrosis-related Diabetes( treated with insulin)
• Male infertility
• Osteoporosis
• Arthropathy
What are the Diagnostic criteria for CF?
**Must to have one symptom from each one.
AND
1- Clinical symptoms consistent with CF in at least one organ system or positive
newborn screen or having a sibling with CF.
2- Evidence of cystic fibrosis transmembrane conductance regulator (CFTR)
dysfunction (any of the following):
A)• Elevated sweat chloride ≥60 mmol/L ( TWICE to confirm)
B) • Presence genetic mutations in the CFTR gene.
