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)
–
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
–
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
–
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)
–
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
