Bronchiectasis and Cystic Fibrosis Flashcards
bronchiectasis
-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
causes of bronchiectasis
-Recurrent inflammation or infection of airways
-Cystic fibrosis
-Lung infection
-Abnormal lung defense mechanisms
-Localized airway obstruction
pathogenesis of bronchiectasis ‘vicious cycle’
-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
classification of bronchiectasis
-shape of malformation
-normal
-varicose
-cylindrical- MC
-cystic
epidemiology bronchiectasis
-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
pathophysiology bronchiectasis
-Induction of bronchiectasis requires 2 factors:
-An infectious insult
-Impaired drainage, airway obstruction, or a defect in host defense
lung infection causing bronchiectasis
-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
signs of symptoms of bronchiectasis
-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
diff dx bronchiectasis
-Chronic obstructive pulmonary disease
-Asthma
-Bronchiolitis
-Allergic bronchopulmonary aspergillosis
bronchiectasis testing
-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)
chest x ray bronchiectasis
-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
high resolution CT bronchiectasis
-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)
frustrating and challenging disease: bronchiectasis
-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
bronchiectasis is different from other pulmonary disease
-Anatomic rather than Physiologic Defect:
-Not amenable to medication
-Lack of funding for research
-cant clear airways due to anatomic defect
most strategies are reactive rather than proactive: bronchiectasis
-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
proactive strategies: bronchiectasis
-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
bronchiectasis: airway clearance therapy
-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
bronchiectasis bronchoscopy
-Evaluate hemoptysis
-Remove retained secretions
-Rule out obstructing airway lesions
bronchiectasis: tx
-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
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)
cystic fibrosis
-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
cystic fibrosis foundations pt registry (US)
-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
cystic fibrosis: clinical features
-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
cystic fibrosis: pulmonary manifestations in adults
-Acute and chronic bronchitis
-Bronchiectasis
-Pneumonia
-Atelectasis
-Peribronchial and parenchymal scarring
-Pneumothorax and hemoptysis
-Hypoxemia, hypercapnia, and cor pulmonale (advanced cases)
cystic fibrosis: signs and symptoms
-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
cystic fibrosis physical
-Digital clubbing
-Increased AP chest diameter
-Hyperresonance to percussion
-Apical crackles
-Sinus tenderness, purulent nasal secretions, nasal polyps
cystic fibrosis diff dx
-COPD
-Asthma
-Alpha 1-Antiprotease deficiency
-Bronchiolitis
-Celiac sprue
-Chronic sinusitis
cystic fibrosis: dx criteria
-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)
cystic fibrosis dx testing
-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
cystic fibrosis: sputum cultures
-Frequent- S aureus and P aeruginosa
-Occasional- H influenzae, S maltophilia, and B cepacia
cystic fibrosis: imaging
-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
-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
cystic fibrosis antibiotics, bronchodilators, vaccine, surgery
-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
cystic fibrosis vs bronchiectasis: obstructive/restrictive
-bronchiectasis- obstructive -> dilated airways collapse distally
-cystic fibrosis- air way affected and fibrotic -> obstructive and restrictive
