Bronchiectasis and Cystic Fibrosis

Question 1

bronchiectasis

Answer 1

-congenital or acquired d/o of the bronchi characterized by permanent, abnormal dilation and impaired clearance of secretions
-Permanent Abnormal Dilatation of the bronchi
-Historically, the most common cause of bronchiectasis is severe lung infections during childhood (e.g. whooping cough, pneumonia, TB)
-More recently, bronchiectasis has been strongly associated with moderate-severe COPD
-The hallmark pulmonary pathology observed in cystic fibrosis is bronchial mucous plugging, inflammation, and eventually bronchiectasis

Question 2

causes of bronchiectasis

Answer 2

-Recurrent inflammation or infection of airways
-Cystic fibrosis
-Lung infection
-Abnormal lung defense mechanisms
-Localized airway obstruction

Question 3

pathogenesis of bronchiectasis ‘vicious cycle’

Answer 3

-Infection
-Release of inflammatory cytokines
-Destruction of mucociliary and cartilagenous supporting structures
-Loss of ventilatory function
-permanently dilation of airways
-Impairment of Mucociliary clearance
-Colonization and biofilm formation

Question 4

classification of bronchiectasis

Answer 4

-shape of malformation
-normal
-varicose
-cylindrical- MC
-cystic

Question 5

epidemiology bronchiectasis

Answer 5

-Prevalence unknown
-An estimated 350,000 to 500,000 adults have bronchiectasis in the US
-Increases with age
-More common in women
-Utilize a tremendous amount of healthcare resources

Question 6

pathophysiology bronchiectasis

Answer 6

-Induction of bronchiectasis requires 2 factors:
-An infectious insult
-Impaired drainage, airway obstruction, or a defect in host defense

Question 7

lung infection causing bronchiectasis

Answer 7

-childhood infections (pertussis, measles) -> dx by hx of infection
-bacterial infection (infections due to staph aureus, pseudomonas aeruginosa) -> dx by hx of infection, sputum
-viral infections (infections due to adenovirus, influenza, herpes) -> dx by hx/serologic evidence of infection
-other infections (fungal, mycobacterium tuberculosis, mycoplasma) -> dx by fungal culture, AFB smear and mycobacterial culture

Question 8

signs of symptoms of bronchiectasis

Answer 8

-Chronic cough with production of copious amounts of purulent sputum**
-Dyspnea and wheezing (75%)
-Hemoptysis
-Pleuritic chest pain
-Weight loss
-Anemia

-Physical findings: nonspecific
-Copious, foul-smelling, purulent sputum is characteristic**
-Persistent crackles at bases common
-squeaking
-Clubbing common in severe disease

Question 9

diff dx bronchiectasis

Answer 9

-Chronic obstructive pulmonary disease
-Asthma
-Bronchiolitis
-Allergic bronchopulmonary aspergillosis

Question 10

bronchiectasis testing

Answer 10

-CBC with Diff
-Immunoglobulins: IgG, IgM, and IgA
-look for abnormal immunodeficiency states
-Sputum stain/culture: bacteria, mycobacteria, fungi
-pseudomonas is a common colonizer!
-look for abnormal secretion clearance
-Sweat test for sodium and chloride levels
-Alpha1-Antiprotease level
-RF
-look for miscellaneous disorders
-PFTS!!!!:
-Obstructive pulmonary dysfunction with hypoxemia (moderate/severe disease)

Question 11

chest x ray bronchiectasis

Answer 11

-Dilated and thickened bronchi* that may appear as “tram-tracks” (parallel lines) or as ring-like markings
-Scattered irregular opacities, atelectasis, and focal consolidation may be present
-honeycombing- cystic bronchiectasis
-chest x ray is not good -> do a CT
-CT is how we dx

Question 12

high resolution CT bronchiectasis

Answer 12

-this is how me make dx
-Airway dilatation: parallel lines(tram-tracks) or ring shadows

-Mucopurulent plugs or debris accompanied by post-obstructive air trapping:
-Peripheral, irregular, short (2 to 4 mm) linear branching markings are noted: “tree-in-bud pattern”

-Cysts off the bronchial wall:
-In heavily involved areas, the cysts are clustered to appear like grapes (cystic bronchiectasis)

Question 13

frustrating and challenging disease: bronchiectasis

Answer 13

-Patients tend to be sick more often than not

-They are either:
-Actively infected
-Just got over an infection
-Just about to get sick again

-They experience:
-Frequent hospitalizations with recurrent lower respiratory infections
-Very high readmission rate

Question 14

bronchiectasis is different from other pulmonary disease

Answer 14

-Anatomic rather than Physiologic Defect:
-Not amenable to medication
-Lack of funding for research
-cant clear airways due to anatomic defect

Question 15

most strategies are reactive rather than proactive: bronchiectasis

Answer 15

-Most common approach to bronchiectasis is treating respiratory infections as they arise (non preventative)
-Patient receive frequent courses of antibiotics both inpatient and outpatient.
-High rate of colonization with resistant organisms including P. aeruginosa, MRSA

Question 16

proactive strategies: bronchiectasis

Answer 16

-primary defect is the inability to clear secretions.
-The primary strategy should be to improve airway clearance.
-Effective airway clearance leads to a dramatic decrease in the rate of infections

Question 17

bronchiectasis: airway clearance therapy

Answer 17

-Manual chest physical therapy (CPT)- not realistic
-(positive expiratory) PEP devices: Acapella, Aerobika -> increase intrathoracic pressure to help clear secretions
-Nebulized saline (NS or Hypertonic)
-Nebulized Bronchodilators
-Mucolytics
-VEST devices- vibration *****
-when we keep airways clear -> prevents sickness
-prioritize this tx if you can - if systemic signs do antibiotics

Question 18

bronchiectasis bronchoscopy

Answer 18

-Evaluate hemoptysis
-Remove retained secretions
-Rule out obstructing airway lesions

Question 19

bronchiectasis: tx

Answer 19

-Acute exacerbations:
-Initial antibiotic regimen tailored to prior sputum cultures and sensitivities (not empiric)
-Multiple prior exacerbations or no recent sputum culture -> Fluoroquinolone antibiotic 7-10 days

-Recurrent exacerbations (2-3/year):
-Preventive therapy with a macrolide -> decrease respiratory secretions!!! (not used for antibiotic effect)

-Bronchodilators- Airflow limitation on spirometry

-Glucocorticoids:
-Inhaled: asthma/copd
-Oral: acute exacerbation

-Vaccinations
-Pulmonary rehab
-Surgical resection:
-For the few pts with localized bronchiectasis and adequate pulmonary function in whom conservative management fails

Question 20

bronchiectasis complications

Answer 20

-Hemoptysis -> Massive hemoptysis may require embolization of bronchial arteries or surgical resection*
-Cor pulmonale
-Secondary amyloidosis
-Secondary visceral abscesses at distant sites (eg, brain)

Question 21

cystic fibrosis

Answer 21

-Most common fatal autosomal recessive ds in Caucasians.
-Abnormalities in a membrane chloride channel (cystic fibrosis transmembrane conductance regulator [CFTR] protein)
-Results in altered chloride transport and water flux across the apical surface of epithelial cells
-thick secretions
-bronchiectasis at young age

Question 22

cystic fibrosis foundations pt registry (US)

Answer 22

-More than 30,000 people are living with cystic fibrosis (more than 70,000 worldwide).
-Approximately 1,000 new cases of CF are diagnosed each year.
-More than 75 percent of people with CF are diagnosed by age 2 due to newborn screening.
-More than half of the CF population is age 18 or older

Question 23

cystic fibrosis: clinical features

Answer 23

-Exocrine glands produce abnormal mucous that obstructs glands and ducts -> Glandular dilation and tissue damage
-Inadequate hydration of the tracheobronchial epithelium
-Impairs mucociliary function
-Increased sputum viscosity

-Biliary cirrhosis, gallstones, pancreatitis
-Congenital bilateral absence of the vas deferens with azoospermia

Question 24

cystic fibrosis: pulmonary manifestations in adults

Answer 24

-Acute and chronic bronchitis
-Bronchiectasis
-Pneumonia
-Atelectasis
-Peribronchial and parenchymal scarring
-Pneumothorax and hemoptysis
-Hypoxemia, hypercapnia, and cor pulmonale (advanced cases)

Question 25

cystic fibrosis: signs and symptoms

Answer 25

-Suspect in young adult with:
-History of chronic lung ds (especially bronchiectasis)
-Pancreatitis/insufficiency
-Infertility

-Cough
-Sputum production
-Decreased exercise tolerance
-Recurrent hemoptysis
-Sinus pain/pressure and purulent nasal discharge
-Steatorrhea, diarrhea, abdominal pain

Question 26

cystic fibrosis physical

Answer 26

-Digital clubbing
-Increased AP chest diameter
-Hyperresonance to percussion
-Apical crackles
-Sinus tenderness, purulent nasal secretions, nasal polyps

Question 27

cystic fibrosis diff dx

Answer 27

-COPD
-Asthma
-Alpha 1-Antiprotease deficiency
-Bronchiolitis
-Celiac sprue
-Chronic sinusitis

Question 28

cystic fibrosis: dx criteria

Answer 28

-Following criteria must be met to diagnose CF
-1. Clinical symptoms consistent with CF in at least one organ system
-2. Evidence of CFTR dysfunction:
-Elevated sweat chloride >60 mmol/L
OR
-genetic testing- Presence of 2 disease-causing mutations in CFTR
-Abnormal nasal potential difference (used if sweat chloride neg)

Question 29

cystic fibrosis dx testing

Answer 29

-Arterial blood gas studies:
-Hypoxemia
-Advanced disease: chronic compensated respiratory acidosis

-Pulmonary function studies:
-Mixed obstructive and restrictive pattern
-Reduction in FVC, airflow rates, and TLC
-Air trapping (high ratio of RV to TLC)

-sputum

CXR

Question 30

cystic fibrosis: sputum cultures

Answer 30

-Frequent- S aureus and P aeruginosa
-Occasional- H influenzae, S maltophilia, and B cepacia

Question 31

cystic fibrosis: imaging

Answer 31

-Chest x-ray
-Hyperinflation (early)
-Peribronchial cuffing
-Mucous plugging
-Bronchiectasis (ring shadows,cysts)
-Increased interstitial markings
-Small rounded peripheral opacities
-Focal atelectasis
-Pneumothorax

Question 32

cystic fibrosis: tx

Answer 32

-Referral to a regional cystic fibrosis center is strongly recommended

-Conventional treatment programs focus on the following areas:
-Clearance and reduction of lower airway secretions
-Reversal of bronchoconstriction
-Treatment of respiratory tract infections and airway bacterial burden
-Pancreatic enzyme replacement
-Nutritional and psychosocial support

-CTFR Modulators***- 3-4 drugs available now, based on pts genotype and age

-Clearance of lower airway secretions
-Postural drainage, chest percussion or vibration techniques, VEST devices*, positive expiratory pressure (PEP) or flutter valve breathing devices
-Inhaled recombinant human deoxyribonuclease (rhDNase) *cleaves extracellular DNA in sputum
-Inhalation of hypertonic saline
-Exercise

Question 33

cystic fibrosis antibiotics, bronchodilators, vaccine, surgery

Answer 33

-Active airway infections based on results of c & s of sputum:
-S aureus (including methicillin-resistant strains)
-P aeruginosa
-H influenzae, Stenotrophomonas maltophilia, and B cepacian
-Chronic tx with Azithromycin (antiinflammatory)

-inhaled bronchodilators: albuterol if responsive -> prior ot physiotherapy

-vaccination- pneumococcal and influenza

-lung transplantation- only definitive tx

Question 34

cystic fibrosis vs bronchiectasis: obstructive/restrictive

Answer 34

-bronchiectasis- obstructive -> dilated airways collapse distally
-cystic fibrosis- air way affected and fibrotic -> obstructive and restrictive