Bronchiectasis

Question 1

• Uncommon
• Results from infectious process
  
  • Leads to permanent distortion of one or more of the conduction airways
• Categorized as COPD due to airway collapse causing obstruction

What disease is this?

Answer 1

Bronchiectasis

Question 2

Etiology of Bronchiectasis

Answer 2

• Cystic Fibrosis (50% of cases)
• Primary infections
• Aspiration
• Congenital anatomic defects
• connective-tissue disorders
• Alpha1-antitrypsin (AAT) deficiency
• Autoimmune disease
• Toxic gas exposure
• Haemophilus Influenza: most common organism in non-CF patients with bronchiectasis
• P. aeruginosa, S. pneumonia, and S. aureus also commonly identified
  
  • Pseudomonas associated with accelerated course, more frequent exacerbations and more rapid decline in lung function

Question 3

Abnormal dilation of proximal and medium-large sized bronchi (>2mm in diameter)

Can be congenital but most often acquired

Infection ->imparied drainage -> airway obstruction

muscular and elastic components of the bronchial wall and often peribronchial alveolar tissue damaged from the inflammatory process

Overall result = retained secretions colonized with pathogenic organisms resulting in copious amounts of purulent sputum

What disease is this the pathology of?

Answer 3

Bronchiectasis

Question 4

What is the vicious cycle of Bronchiectasis?

Answer 4

• Infection: with acute inflammation and recruitment of inflammatory cells
• Release: of inflammatory cytokines peroxidases, proteinases elastase
• Destruction: of mucociliary and cartilagenous supporting structure
• Loss: of ventilatory function
• Impariment: mucociliary clearance sputum retention

Question 5

Bronchiectasis: Presentation

Answer 5

• Cough and daily mucopurulent sputum lasting months to years
• Blood-streaked/hemoptysis due to airway damage
• Pleuritic chest pain
• dyspnea
• wheezing
• weight loss
• fever
• Crackles/rhonchi with infection
• May have scattered wheezing due to secretions in airways
• clubbing
• cyanosis, wasting

Question 6

Bronchiectasis: Work up

Answer 6

• ***GOLD STANDARD: High resolution CT (HRCT)
• Sputum Analysis: may reveal Dittrich plugs (small white/yellow concentrations)
• Gram stain & culture: may reveal Pseudomonas or E.coli
• Plain CXR obtained in all patients: increased pulmonary markings, ring-like structures, atelactasis, dilated and thickened airways (tram lines), mucus plugging (finger-in-glove) appearance.
• OR
• CXR MAY BE NORMAL
• CBC: Leukocytosis with left shift, polycythemia can be present due to chronic hypoxia
• Quantitative serum alpha1-antitrypsin level to r/o AAT deficiency
• Pilocarpine iontophoresis (sweat test) to r/o cystic fibrosis
• Autoimmune screening

Question 7

Bronchiectasis Diagnostic Evaluation

Answer 7

Spirometry:

obstructive pattern

reduced or normal forced FVC (may be low in advanced dz)

Low FEV1

Low FEV1/FVC

Question 8

Bronchiectasis Management

Answer 8

• improve symptoms
• reduce complications
• control exacerbations
• reduce morbidity and mortality
• antibiotics and chest physiotherapy
• bronchodilators
• corticosteroids
• oxygen
• surgical intervention
• Smoking cessation is essential
• adequate nutrition: pts w/CF
• immunizations (influenza/pneumococcus)
• all other immunizations UTD

Question 9

What is the mainstay of treatment for Bronchiectasis?

Answer 9

Antibiotics

**In acute exacerbations use broad-spectrum abx

Question 10

Acceptable outpt choices for antibiotics:

Answer 10

• amoxicillin
• tetracycline
• trimethoprim-sulfamethoxazole
• macrolide (azithromycin or calrithromycin)
• second-generation cephalosporin
• fluoriquinolone
• Severe illness requiring hospitalization
  
  • IV abx

Question 11

What is one of the most important parts of treatment fro bronchiectasis?

Answer 11

• bronchial hygeine
• postural drainage with percussion and vibration loosens and mobilizes secreations
• Devices: flutter device, intrapulmonic percussive ventilation devices, and incentive spirometry
• “Vest” system is relatively new pneumatic compression device/vest work periodically throughout the day
• Nebulizers with concentrated (7%) sodium chloride solution beneficial

Question 12

Management for severe disease

Answer 12

• lung resection
• transplant