Chronic respiratory disease

Question 1

COPD pathophysiology

Answer 1

Walls of alveoli are destroyed and bronchioles lose their structure and collapse –> tissue destruction, air trapping, excessive sputum

Chest hyperinflation from air trapping –> diaphragm flattening –> worse length tension relationship –> less efficient ventilation

Question 2

COPD defenition

Answer 2

Group of chronic respiratory diseases characterized by progressive tissue degermation –> obstruction of airway
- Emphysema
- Chronic bronchitis

Question 3

COPD risk factors

Answer 3

• Tabaco smoking
• air pollution
• Occupational exposure (dust, fumes)
• Genetic (alpha 1 antitrypsin)
• Age & sex (older and female)
• SEC
• Asthma
  Chronic respiratory infections

Question 4

COPD Clinical features

Answer 4

Chronic and progressive dyspnea, cough, sputum production
Accessory muscle use, pursed lip breathing
Increased A-P chest diameter
Central cyanosis (skin and mucus appear blue/purple)
Digital clubbing

Question 5

COPD Diagnosis

Answer 5

PFT - FEV1/FVC <70 to confirm airflow limitation
Sputum culture, ABGs, CT scam
Based on symptoms and exposure to risk factors

Question 6

Emphysema etiology

Answer 6

Genetic deficiency of alpha 1 antitrypsin (inhibits proteases that are destructive enzymes released during inflammation)
Cigarettes smoking (increases neutrophils and decrease effect of A1A)
Infection
Air pollutants

Question 7

Emphysema pathophysiology

Answer 7

Destruction of alveolar walls and septae –> bullae (permanently inflated alveolar space) –>
- Loss of SA and pulmonary capillaries
- Fibrosis and thickening of bronchiole walls due to chronic irritation and infections limiting air flow and increasing mucus production
- Loss of tissue elasticity
- Collapse of small bronchi during expiration
- Gas trapping (behind bronchioles), increased residual volume, increase P-A diameter & hyperinflation
Advanced emphysema –>
- Bullae at risk of rupturing causing pneumothorax
- Hypercapnia and hypoxic drive to breathe
- Infection
Pulmonary hyper tension and cor pulmonale

Question 8

Emphysema clinical factors

Answer 8

Insidious onset
Dyspnea on exertion & later at rest
Hyperventilation with prolonged expiratory phase& accessory muscle
Hyper resonant on percussion
Weight loss
Clubbed finger

Question 9

Emphysema diagnosis

Answer 9

CXR
PFT - increased residual volume and TLC, decreased VEC and VC

Question 10

COPD Treatment and prognosis

Answer 10

Goals: control symptoms, reduce exasperations, improve QOL

Smoking cessation
Pharmacological
- Maintenance: bronchodilators, mucolytic expectorants
- Acute: oral corticosteroids, antibiotics
Assessment of inhaler technique
Long term oxygen therapy
- 15hrs a day if chronically hypoxemic
- Oxygen concentrator takes in air and filters other gases to getO2
Surgery
- lung volume reduction: removes emphysematous tissue or 1 way valve in damaged area to reduced V/Q mismatch
- lung transplant: end state of COPD
Physio:
- pulmonary rehab
- dyspnea management
○ Breathing techniques, positioning
- Airway clearance
Increase exercise tolerance

Progressive chronic condition with acute perturbation

Question 11

Emphysema treatment and prognosis

Answer 11

Avoid respiratory irritants and source of infection
Immunization against pneumonia and influenza
Adequate nutrition and hydration
Meds
LVR surgery to reduce air trapping
Physio:
- pulmonary rehab
- dyspnea management
○ Breathing techniques, positioning
- Airway clearance
- Increase exercise tolerance

Respiratory failure due to sever hypoxia or hypercapnia
Cor Pulmonale
Progressive

Question 12

Chronic bronchitis patho

Answer 12

Changes in bronchi from chronic irritation from smoking or exposure to pollutants
Inflamation, obstruction, repeated infection & coughing –>
- Mucosa inflamed and edematous
- Increased mucus secretion
- Bronchial wall thickening–> difficult clearing secretions
- Hypoxia
- Severe dyspnea and fatigue
Pulmonary hypertension

Question 13

Chronic bronchitis etiology

Answer 13

Cigarette smoking, environmental pollutants

Question 14

Chronic bronchitis clinical features

Answer 14

Chronic cough with sputum
Tachypnea, shortness of breath
Airway obstruction –> hypoxia, cyanosis and hypercapnia
Weight loss
Secondary polycythemia
- Compensatory response to hypoxia causing increase in RBC, blood thickening
Increased thrombosis, stroke and right ventricle enlargement and failure (cor pulmonale)

Question 15

Chronic bronchitis diagnosis

Answer 15

Based on assessment
Productive cough lasting 3 months of the year for 2 consecutive years

Question 16

Chronic bronchitis treatment and prognosis

Answer 16

Avoid respiratory irritants
Prompt treatment of infection
Immunization against pneumonia and influenza
Bronchodilators, expectorants
Meds
LVR surgery to reduce air trapping
Physio: Airway clearance

Question 17

Asthma definition

Answer 17

Chronic disorder characterized by reversible airflow obstruction and airway inflammation, persistent airway hyperactivity and airway remodeling

Question 18

Asthma etiology

Answer 18

Genetics
Maternal factors (young, smoking)
Viral upper respiratory infection
Sedentary, often indoors
Air pollution
Exercise induced asthma

Question 19

Asthma patho

Answer 19

Regardless of trigger, bronchi and bronchioles respond to stimuli w/
- Airway inflammation
- Bronchospasm/constriction
- Increase mucus secretion
This leads to partial or complete obstruction –> decreased airflow, air trapping, lung hyperinflation.
Sputum blocks flow of air –> non aeration (atelectasis)
Thickening of bronchiole walls
Fibrous tissue from chronic inflammation

Question 20

Asthma clinical features

Answer 20

Wheeze
Cough (often worse at night)
SOB
Chest tightness
Signs of obstruction
- Increased RR, use of accessory muscles
Tachycardia and/or pulse paradox (HR differs on inspiration and expiration)
Hypoxia

Question 21

Asthma diagnosis

Answer 21

PFTs and history
Variable airflow limitation or hyper responsiveness to triggers
Airflow limitation that reverses with bronchodialato

Question 22

Asthma treatment and prognosis

Answer 22

Goals: control symptoms, reduce exasperations

Pharmacological
- Acute: : bronchodilators
- inhaled corticosteroids,
Education
- Assessment of inhaler technique, action plan, peak flow monitoring
Physio:
- dyspnea management
○ Breathing techniques, positioning
Increase exercise tolerance

Can be well managed
Adult onset more likely to become life long
Not progressive

Question 23

Bronchiectasis

Answer 23

Irreversible abnormal dilation primarily of medium sized bronchi

Question 24

Bronchiectasis patho

Answer 24

Requires 2 factors:
- Infectious insult
- Impaired airway drainage, airway obstruction or reduced host defense
Host response created inflammation and mucosal edema
Persistent inflammation and neutrophil presence results in progressive airway destructions –> weakening of muscle and elastic fibres in bronchial wall
Fibrous adhesions form and dilate bronchial wall
Fluid accumulates in dilated area and becomes infected
Infection causes loss of cilia, more fibrosis and progressive obstructi

Question 25

Bronchiectasis etiology

Answer 25

Infectious trigger Airway obstruction (e.g foreign bodies) Anatomical defects Poor host defense CF, asthma Pulmonary infections Systemic disease

Question 26

Bronchiectasis clinical features

Answer 26

Chronic cough and production of purulent sputum Dyspnea, wheeze, hemoptysis Chest pain, fever Weight loss, fatigue

Question 27

Bronchiectasis diagnosis

Answer 27

Cough on most days with tenacious sputum One or more exasperations a year Bronchial airway dilation on CT scan

Question 28

Bronchiectasis treatment and diagnosis

Answer 28

Medical - Bronchodilators, antibiotics Physio - Airway clearance - Exercise Pulmonary rehab Normal life expectancy Progressive but can be managed

Question 29

Cystic fibrosis definition

Answer 29

Multi system disorder arising from mutation of CFTR gene on 7th chromosome cause defect in exocrine gland

Question 30

cystic fibrosis etiology

Answer 30

Autosomal recessive disorder from CTFR gene mutation Many carriers

Question 31

Cystic fibrosis patho

Answer 31

CFTR mutation --> decreased secretion of chloride and increase Na reabsorption --> reabsorption of water resulting in thicker mucus on epithelial lining and more viscous secretions Thickened secretions affect sinuses, lungs, hepatic system, intestine, sweat gland and reproductive system Sinus - increased secretion viscosity impairs cilia, increased bacterial colonization and infections Sweat gland - Increased sodium loss via sweat Lungs - secretions obstruct airflow causing air trapping and atelectasis Stagnant secretions --> infections Obstruction --> respiratory failure or cor pulmonale

Question 32

Cystic fibrosis diagnosis

Answer 32

Newborn screening Sweta chloride test Genetic test

Question 33

Cystic fibrosis clinical features

Answer 33

In kids - Meconium ileus at birth - Respiratory symptoms - Failure to thrive - malnutrition Common sx - Sinusitis - Persistent productive cough - Pancreatic insufficiency - Ileal obstruction - MSK disorder (less BMD) - Malnutrition - Infertility (males) Decreased fertility (female)

Question 34

Cystic fibrosis treatment and prognosis

Answer 34

Interdisciplinary approach Meds - CTFR modulators that improve CTFR function - Inhaled airway clearance agents - Bronchodilators - Anti inflammatory - Antibiotic for acute exasperations Allied professionals - Pancreatic enzyme replacement - Heart/lung transplant - Prevention. And aggressive treatment of infections Physio - Airway clearance Exercise Respiratory failure Life expectancy around 50 years

Question 35

Interstitial lung disease

Answer 35

Group of conditions that causes inflammation and scarring of lungs

Question 36

Interstitial lung disease patho

Answer 36

Often begin in interstitial but also associated with alveolar and airway structure

Question 37

Interstitial lung disease etiology

Answer 37

Idiopathic Exposure related - Environment or occupational pollutants - Meds - Radiation Autoimmune related (e.g. connective tissue)

Question 38

Idiopathic pulmonary fibrosis

Answer 38

Idiopathic specific form of chronic fibrosis interstitial pneumonia limited in the lung

Question 39

IPD patho

Answer 39

Inflammatory cells are released when lungs are injured releasing reactive oxygen species, cytokines, growth factor --> fibrosis This alters lung structure and mechanical properties --> restriction and decreased gas exchange Inflammation also affects alveoli, airway and circulation --> more dyspnea on exertion, decreased exercise tolerance and deconditioning

Question 40

IPF clinical features

Answer 40

Gradual onset of dyspnea and non productive cough over month Dry cough Extreme fatigue, loss of weight and appetite Weakness

Question 41

IPF Diagnosis

Answer 41

Based on clinical features Biopsy to look at histopathalogical pattern

Question 42

Sarcoidosis

Answer 42

Multisystem disorder of unknown etiology characterized by abnormal inflammatory cells that form lungs called granulomata

Question 43

Sarcoidosis clinical features

Answer 43

Cough, dyspnea, chest pain, eye or skin lesion

Question 44

Sarcoidosis diagnosis

Answer 44

Clinical and radiograph signs Excluding other illnesses histopathalogical detection of non-necrotizing granuloma

Question 45

Pneumonitis

Answer 45

General term describing inflammation of lung tissue caused by irritant

Question 46

Pneumonitis etiology

Answer 46

Airbourne irritant or medications that cause body to mount strong immune response Working in grain or wood warehouse

Question 47

Pneumonitis clinical features

Answer 47

Difficulty breathing, dy cough, fatigue, loss of appetite, fever

Question 48

Pneumonitis diagnosis and prognosis

Answer 48

Clinical exam, allergy blood test, bronchoscopy, CT, lung biopsy Long term exposure or insufficient treatment can cause fibrosis and pulmonary hypertension