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Respiratory system > S6: asthma, COPD, bronchiectasis & cystic fibrosis > Flashcards

S6: asthma, COPD, bronchiectasis & cystic fibrosis Flashcards

1
Q

What is asthma?

A

Chronic inflammatory disorder of the airways
Inflammatory symptoms are usually associated with widespread but variable airflow obstruction and an increase in airway responsiveness to a variety of stimuli
Characterised by a triad of bronchial smooth muscle contraction, airway inflammation & increased secretions

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2
Q

Describe the difference in reversibility after bronchodilator use in asthma and COPD

A

Asthma: airway obstruction in asthma is often reversible – improvement in FEV1 of 12% or more after bronchodilators
COPD: airways obstruction is not fully reversible - < 12% improvement FEV1 on spirometry after bronchodilators

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3
Q

Describe the pathophysiology of asthma

A

Initially a type 1 hypersensitivity reaction (immediate response)
Allergen -> dendritic cell -> CD4+ cells -> Th2 cell
Th2 cells activate B-lymphocytes (also activated by re-exposure of allergen) & eosinophils
B-lymphocytes -> plasma cells -> IgE -> mast cells have receptor (Fc)
Mast cell degranulation – histamine, cytokines, leukotrienes & eosinophils secrete leukotrienes & cytokines -> these both cause inflammation and bronchoconstriction

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4
Q

List asthma triggers

A 
Allergens – pollen, animals
Cold air
GORD
Exercise
Fumes – car exhaust 
Cigarette smoke
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5
Q

Describe what long term poorly controlled asthma can lead to

A

Airway remodelling, some of which may not be fully reversible
Changes include:
-hypertrophy & hyperplasia of smooth muscle
-hypertrophy of mucus glands
-thickening of basement membrane

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6
Q

Describe the effect of airways narrowing in asthma

A

Causes wheezing & other clinical features of asthma
Results in an obstructive pattern on spirometry & typical flow volume loops
Air trapping with increased residual volume & hence increased functional reserve capacity

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7
Q

Describe the effect on gas exchange in asthma

A

Airway narrowing -> reduced ventilation of the affected alveoli -> V/P mismatch
In unmanaged mild to moderate asthma: decreased pCO2 & pO2 = type 1 respiratory failure
Severe attacks: extensive involvement of airways with complete blockage of some airways & exhaustion (limits respiratory effort) -> limits the amount of CO2 which can be breathed out
Increasing pCO2 is a sign of LIFE-THREATENING ASTHMA

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8
Q

Describe signs and symptoms of asthma

A 
Dry, nocturnal cough
Wheeze
Breathlessness
Chest tightness 
Atopy
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9
Q

Describe the management of asthma

A

Step 1: short acting beta-2 agonist (finger tremor is a side effect), bronchial smooth muscle relaxation (blue inhaler)
Inhaled corticosteroid – anti-inflammatory (brown inhaler)
Step 2: combination inhaler – long acting beta-2 agonist & anti-inflammatory
Step 3: can increase dose of inhaled corticosteroid or add leukotriene receptor antagonist
Step 4: specialist care

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10
Q

Describe the emergency management of asthma

A

Oxygen
Short acting beta-2 agonist
Steroids
ADMIT

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11
Q

What is COPD?

A

A disease characterised by persistent respiratory symptoms & airflow limitation
Due to airways and/or alveolar abnormalities
Caused by significant exposure to noxious particles or gases

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12
Q

Describe the pathophysiology of COPD

A

Small airways disease – airway inflammation, airway fibrosis, luminal plugs
Parenchymal destruction – loss of alveolar attachments, decrease of elastic recoil
Both lead to airflow limitation

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13
Q

Describe emphysema and chronic bronchitis

A

Emphysema – elastin breakdown & subsequent loss of alveolar integrity leading to permanent destructive enlargement of the airspaces distal to the terminal bronchioles
Chronic bronchitis – excessive mucous secretion and impaired removal of the secretions

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14
Q

Describe the diagnosis of COPD

A

Symptoms: shortness of breath, chronic cough & sputum
Risk factors: host factors, tobacco, occupation & indoor/outdoor pollution
Spirometry: required to establish diagnosis

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15
Q

List the signs of COPD

A 
Often few or none especially at rest
Purse lip breathing
Hyperinflation/barrel-shaped chest 
Prolonged expiratory phase 
Wheeze on auscultation 
Late features include cyanosis & right-sided heart failure
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16
Q

Describe the investigations for COPD

A

Spirometry shows an obstructive pattern with FEV1/FVC ratio <70% & limited reversibility following treatment with bronchodilators
Decreased diffusing capacity of the lung for CO is a feature of emphysema

17
Q

Describe management/treatment for COPD

A 
Smoking cessation
Vaccinations 
Pulmonary rehabilitation 
Long term oxygen treatment 
Surgical interventions
18
Q

List complications of COPD

A

Recurrent pneumonia
Pneumothorax
Respiratory failure
Cor pulmonale

19
Q

What is bronchiectasis?

A

Chronic, irreversible dilatation of one of more bronchi due to destruction of the elastic and muscular components of the bronchial wall
Bronchi exhibit poor mucous clearance and there is predisposition to recurrent or chronic bacterial infections

20
Q

List the causes of bronchiectasis

A 
Post-infection
Immune deficiency
Genetic/mucociliary clearance defects – cystic fibrosis 
Obstruction
May co-exist with COPD
21
Q

Describe the symptoms and signs of bronchiectasis

A 
Cough
Sputum production 
Crackles, high-pitched inspiratory squeaks & rhonchi 
Dyspnoea 
Fever
22
Q

Differentiate COPD from bronchiectasis

A

Diminished breath sounds in COPD
Bronchiectasis – rhonchi may be auscultated, but with additional inspiratory squeaks and crackles
Chest CT – normal/show emphysema in COPD, thickened, dilated airways with/without air fluid levels in bronchiectasis

23
Q

Differentiate bronchiectasis from asthma

A

Inspiratory squeaks and crackles are not present in asthma & wheezing not present in bronchiectasis
Chest CT – signet ring sign in bronchiectasis
Bronchiectasis will not be fully reversible with bronchodilator treatment

24
Q

What is the signet ring sign?

A

Seen in bronchiectasis
Dilated bronchus & accompanying pulmonary artery are seen in cross section
Bronchus is markedly dilated and larger than the artery

25
Q

Describe the management of bronchiectasis

A

Treat underlying cause to prevent disease progression
Physiotherapy
Antibiotics
Vaccinations

26
Q

What is cystic fibrosis?

A

Autosomal recessive disorder
Caused by gene mutation on chromosome 7
Abnormal function of the epithelial chloride channel – cystic fibrosis transmembrane conductance regulator (CFTR) – key role in maintaining lung epithelial function

27
Q

Describe the role of CFTR in lungs

A

Transports chloride out of the cell & regulates ENaC
Critical role in hydration of surface mucous airways – defective ion transport reduces airway surface liquid hydration & leads to thick and sticky mucous (impairs muco-ciliary clearance)

28
Q

Describe how CF is diagnosed

A

Positive newborn screening test result/history of CF in a sibling/one or more characteristic phenotypic features
AND increased sweat chloride concentration (sweat test) or identification of two CF mutations (genotyping)

29
Q

List the main CF clinical presentations

A

1) Meconium ileus – infants bowel blocked by sticky secretions
2) Intestinal malabsorption
3) Recurrent chest infections
4) Newborn screening
5) Atypical presentation – mutations that cause faulty, but not entirely misfunctioning, CFTR protein

30
Q

List the clinical features of CF

A 
Chronic sinusitis 
Repeated lower respiratory tract infection 
Nasal polyps 
Pancreatic insufficiency
Male infertility 
Finger clubbing 
Osteoporosis
31
Q

Describe CF lifestyle advice

A 
No smoking
Avoid other CF patients
Avoids friends with infections
Vaccination 
Ensure optimal nutrition
32
Q

List the common CF complications

A

Respiratory infections
Low body weight
Distal intestinal obstruction syndrome – faecal obstruction in ileo-caecum

Respiratory system (11 decks)

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