bronchiectasis + cystic fibrosis Flashcards
bronchiectasis definition and MC presentation
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
causes of bronchiectasis + pathology of CF to bronchiectasis
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
pathogenesis of bronchiectasis ‘vicious cycle’
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
pathophysiology bronchiectasis: what are the two requirements
Need two requirements:
1: Infectious insult
1: Impaired drainage, airway obstruction, or defect in host defense
lung infection causing bronchiectasis
Common Organisms:
- H. influenzae: MC non-cystic fibrosis organism
- Pseudomonas aeruginosa, Strept pneumoniae, and s. aureus also very common
signs + symptoms of bronchiectasis
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
bronchiectasis testing
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
PFTS: bronchiectasis
Obstructive pulmonary dysfunction with hypoxemia (moderate/severe disease)
chest x ray bronchiectasis
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
high resolution CT bronchiectasis
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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Tx bronchiectasis: main struggles (2) ; why is this ds so frustrating?
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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bronchiectasis: tx mc approach
Most common approach: Treat the underlying infections as they arise (non-preventatitve)
-High rate of colonization with resistant organisms including P. aeruginosa, MRSA
proactive strategies: bronchiectasis
Primary strategy: improve airway clearance
-Effective airway clearance leads to a dramatic decrease in the rate of infections
bronchiectasis: airway clearance therapy
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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bronchiectasis bronchoscopy
-To evaluate hemoptysis
-Removes retained secretions
-R/o obstructing airway lesions
bronchiectasis: exacerbation abx tx
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)
bronchiectasis: tx
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
bronchiectasis complications
-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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cystic fibrosis description + pathophysiology
-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
cystic fibrosis: what damage does it cause + effects on lung
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
cystic fibrosis: effects on GI and reproductive system
GI effects:
-Biliary cirrhosis
- gallstones
- pancreatitis
Reproductive effects:
-Congenital bilateral absence of the vas deferens with azoospermia -> INFERTILITY
cystic fibrosis: pulmonary manifestations in adults - what can it cause
-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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cystic fibrosis: signs and symptoms
Chronic cough
Sputum production
Sinus pain/pressure + purulent discharge
Steatorrhea, diarrhea, abd pain
- Meconium ileus is pathognomonic for CF
Decreased exercise tolerance
Recurrent hemoptysis
who should you suspect with CF and what signs?
Young adults with:
- Hx chronic lung disease
- Pancreatitis/Pancreatic Insufficiency
- Infertility
-chronic cough with sputum production
cystic fibrosis physical
-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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cystic fibrosis: dx 2 criteria, what are they
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
cystic fibrosis abg + pft
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
cystic fibrosis: sputum cultures
-Frequent- S aureus and P aeruginosa
-Occasional- H influenzae, S maltophilia, and B cepacia
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cystic fibrosis: Chest x-ray
-Hyperinflation (early)
-Peribronchial cuffing
-Mucous plugging
-Bronchiectasis (ring shadows,cysts)
-Increased interstitial markings
-Small rounded peripheral opacities
-Focal atelectasis
-Pneumothorax
cystic fibrosis: tx
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
Clearance and reduction of lower airway secretions: cystic fibrosis ; what techniques
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
cystic fibrosis tx
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
Definitive tx of CF
-lung transplantation- only definitive tx
CF Sputum Cultures what are the frequent organisms?
–S aureus and P aeruginosa (MC)
Also:
-H influenzae, Stenotrophomonas maltophilia, and B cepacian
cystic fibrosis antibiotics tx
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)
cystic fibrosis vs bronchiectasis: obstructive/restrictive
-bronchiectasis- obstructive -> dilated airways collapse distally
-cystic fibrosis- air way affected and fibrotic -> obstructive and restrictive
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