bronchiectasis + cystic fibrosis

Question 1

bronchiectasis definition and MC presentation

Answer 1

Definition:
- congenital or acquired disorder of the bronchi
- characterized by PERMANENT and abnormal dilation of bronchi with impaired clearance of secretions
-MC: women, older age, COPD
-strongly associated between brochiectasis+ moderate-severe COPD

Question 2

causes of bronchiectasis + pathology of CF to bronchiectasis

Answer 2

Recurrent inflammation or infection of airways:
- severe childhood lung infection* (MC)
-Cystic fibrosis (50%)
-Abnormal lung defense mechanisms
-Localized airway obstruction

Cystic fibrosis pathology:
-bronchial mucous plugging -> inflammation -> bronchiectasis

Question 3

pathogenesis of bronchiectasis ‘vicious cycle’

Answer 3

Pulmonary Infection → Release of Inflammatory Cytokines → Destruction of mucociliary/cartilagenous supporting structures → Loss of Ventilation & Mucociliary clearance function → Build up of mucus → Pathogen colonization → Pulmonary Infection

Question 4

pathophysiology bronchiectasis: what are the two requirements

Answer 4

Need two requirements:
1: Infectious insult
1: Impaired drainage, airway obstruction, or defect in host defense

Question 5

lung infection causing bronchiectasis

Answer 5

Common Organisms:
- H. influenzae: MC non-cystic fibrosis organism
- Pseudomonas aeruginosa, Strept pneumoniae, and s. aureus also very common

Question 6

signs + symptoms of bronchiectasis

Answer 6

sx:
- CHRONIC cough with production of COPIOUS amounts of PURULENT SPUTUM**
-Dyspnea and wheezing (75%)**
-Pleuritic chest pain
-wt loss
-Anemia
-Hemoptysis

Physical findings: nonspecific
-COPIOUS, FOUL-smelling, PURULENT sputum is characteristic**
-Persistent crackles at bases common
-squeaking
-Clubbing: in severe disease

Question 7

bronchiectasis testing

Answer 7

Look for miscellaneous disorders:
-CBC with Diff (infection)
-Immunoglobulins: IgG, IgM, and IgA (look for abnormal immunodeficiency)
-Sputum stain/culture: bacteria, mycobacteria, fungi
-pseudomonas is a common colonizer!
-look for abnormal secretion clearance
-Sweat test for sodium and chloride levels (r/o CF)
-Alpha1-Antiprotease level (emphysema)
-RF

Question 8

PFTS: bronchiectasis

Answer 8

Obstructive pulmonary dysfunction with hypoxemia (moderate/severe disease)

Question 9

chest x ray bronchiectasis

Answer 9

chest x ray is not good -> do a CT
CT = definitive dx

Xray:
- Dilated and thickened bronchi: Appears like tram-tracks or ring shadows
-Scattered irregular opacities, atelectasis, and focal consolidation may be present
-honeycombing = CYSTIC bronchiectasis

Question 10

high resolution CT bronchiectasis

Answer 10

DX GOLD STANDARD
-Airway dilatation: parallel linesor 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”
-cylindrical
-varicose
- cystic

Cysts off bronchial wall
-In heavily involved areas, the cysts are clustered to appear like grapes (cystic bronchiectasis)
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Question 11

Tx bronchiectasis: main struggles (2) ; why is this ds so frustrating?

Answer 11

ANATOMIC DEFECT not physiologic defect:
-cannot cure it with medicine

Patients tend to be sick more often than not
- usually sick
-Frequent hospitalizations with recurrent lower respiratory infections
-Very high readmission rate
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Question 12

bronchiectasis: tx mc approach

Answer 12

Most common approach: Treat the underlying infections as they arise (non-preventatitve)
-High rate of colonization with resistant organisms including P. aeruginosa, MRSA

Question 13

proactive strategies: bronchiectasis

Answer 13

Primary strategy: improve airway clearance
-Effective airway clearance leads to a dramatic decrease in the rate of infections

Question 14

bronchiectasis: airway clearance therapy

Answer 14

First line tx!! better than abx
Goal: improve airway clearance
-VEST devices- best but $$ *****
-Manual chest physical therapy (CPT)- not realistic bc needs therapist
-(positive expiratory) PEP devices: Acapella, Aerobika -> increase intrathoracic pressure to help clear secretions
-Nebulized saline
-Nebulized Bronchodilators
-Mucolytics

-when we keep airways clear -> prevents sickness
-prioritize this tx if you can - if systemic signs do antibiotics
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Question 15

bronchiectasis bronchoscopy

Answer 15

-To evaluate hemoptysis
-Removes retained secretions
-R/o obstructing airway lesions

Question 16

bronchiectasis: exacerbation abx tx

Answer 16

Acute exacerbations: abx tailored to sputum culture

Multiple prior exacerbations or no recent sputum:
- Fluoroquinolones x 7 days

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

Question 17

bronchiectasis: tx

Answer 17

For all:
- Airway Clearance Therapy **
- Bronchodilators (esp if any obstructive pattern)
- inhaled glucocorticosteroids (ICG: oral for acute exacerbation)
- Vaccinations
- Pulmonary rehab

Surgical resection:
-For the few pts with localized bronchiectasis with adequate pulmonary function but failed with conservative management

Question 18

bronchiectasis complications

Answer 18

-Hemoptysis -> Massive hemoptysis may require embolization of bronchial arteries or surgical resection*
-Cor pulmonale
-Secondary amyloidosis
-Secondary visceral abscesses at distant sites (eg, brain)
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Question 19

cystic fibrosis description + pathophysiology

Answer 19

-MC fatal autosomal recessive ds in Caucasians

Pathophysiology
-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
-causes a thick sticky mucus -> impaired mucociliary clearance!!!!

fun facts:
-More than 75 percent of people with CF are diagnosed by age 2 due to newborn screening
- CF PATIENTS PRESENT AT VERY YOUNG AGE
-More than half of the CF population is age 18 or older

Question 20

cystic fibrosis: what damage does it cause + effects on lung

Answer 20

CF is not exclusive to lungs:
- Exocrine glands throughout body produce abnormal mucus that obstructs glands and ducts = Glandular dilatation & Tissue damage

Lung effects:
-Inadequate hydration of the tracheobronchial epithelium
-Impairs mucociliary function
-Increased sputum viscosity

Question 21

cystic fibrosis: effects on GI and reproductive system

Answer 21

GI effects:
-Biliary cirrhosis
- gallstones
- pancreatitis

Reproductive effects:
-Congenital bilateral absence of the vas deferens with azoospermia -> INFERTILITY

Question 22

cystic fibrosis: pulmonary manifestations in adults - what can it cause

Answer 22

-Acute and chronic bronchitis
-Bronchiectasis
-Pneumonia
-Atelectasis
-Peribronchial and parenchymal scarring
-Pneumothorax and hemoptysis
-Hypoxemia, hypercapnia, and cor pulmonale (advanced cases)
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Question 23

cystic fibrosis: signs and symptoms

Answer 23

Chronic cough
Sputum production
Sinus pain/pressure + purulent discharge
Steatorrhea, diarrhea, abd pain
- Meconium ileus is pathognomonic for CF

Decreased exercise tolerance
Recurrent hemoptysis

Question 24

who should you suspect with CF and what signs?

Answer 24

Young adults with:
- Hx chronic lung disease
- Pancreatitis/Pancreatic Insufficiency
- Infertility
-chronic cough with sputum production

Question 25

cystic fibrosis physical

Answer 25

-Sinus tenderness
- purulent nasal secretions
- nasal polyps** (DDx with asthma)
-Digital clubbing: chronic hypoxemia
-Hyper-resonance to percussion** -Increased AP chest diameter
-Apical crackles

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Question 26

cystic fibrosis: dx 2 criteria, what are they

Answer 26

1:Clinical symptoms consistent with CF

2: Evidence of CFTR dysfunction:
- ↑ Sweat Cl- > 60 mmol/L
- Presence of 2+ disease-causing mutations in CFTR gene

If above is normal: Nasal Potential Difference test
- 1 Electrode placed in inferior turbinate and another electrode placed in subcutaneous forearm
- Positive test: Voltmeter reading of > -40 mV

Question 27

cystic fibrosis abg + pft

Answer 27

ABG:
- Early ds: Hypoxemia
-Advanced disease: chronic respiratory ACIDOSIS with compensation**

PFT
-MIXED obstructive and restrictive pattern with air trapping
- ↓ FVC, ↓ TLC: Restrictive
- ↑ RV/TLC ratio (air trapping): Obstructive- lots of air remains in the lung after exhalation
- ↓ Airflow rates (FEV1): obstructive, can’t expell out air because of obstruction

Question 28

cystic fibrosis: sputum cultures

Answer 28

-Frequent- S aureus and P aeruginosa
-Occasional- H influenzae, S maltophilia, and B cepacia
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Question 29

cystic fibrosis: Chest x-ray

Answer 29

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

Question 30

cystic fibrosis: tx

Answer 30

Recommended to refer to cystic fibrosis center

Goal of Therapy:
- Clearance and reduction of lower airway secretions
- Reversal of bronchoconstriction
- Treatment of respiratory tract infections and airway bacterial burden
- Pancreatic enzyme replacement
- Nutritional & Psychosocial support

Question 31

Clearance and reduction of lower airway secretions: cystic fibrosis ; what techniques

Answer 31

Postural drainage
Chest percussion/vibration techniques
VEST devices
PEP devices
Flutter valve breathing device
Inhalation of hypertonic saline
Exercise

Bronchiectasis clearance:

• Chest percussion/vibration techniques
• PEP
• nebulized saline
• bronchodilators
• mucolytics
• VEST
• mucolytics

Question 32

cystic fibrosis tx

Answer 32

Referral- CF specialist
Conventional tx programs
CTFR Modulators: TREAT CAUSE OF DISEASE
- Induce functionality or formation of CFTR channels
- drug: IVACAFTOR*
- Definitive tx: lung transplant

-inhaled bronchodilators: albuterol PRN
-vaccination- pneumococcal and influenza

Question 33

Definitive tx of CF

Answer 33

-lung transplantation- only definitive tx

Question 34

CF Sputum Cultures what are the frequent organisms?

Answer 34

–S aureus and P aeruginosa (MC)

Also:
-H influenzae, Stenotrophomonas maltophilia, and B cepacian

Question 35

cystic fibrosis antibiotics tx

Answer 35

Antibiotic Treatment of underlying infection
- based on results of culture + sensitivity of sputum:

Chronic Treatment: Azithromycin (macrolide)
- for anti-inflammatory effects
- CI with non-TB mycobacteria (risk of resistance)

Question 36

cystic fibrosis vs bronchiectasis: obstructive/restrictive

Answer 36

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