L18. Obstructive and Restrictive Lung Disease Flashcards Preview

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What clinical commonalities do the diverse ranges of lung pathologies share?



What is the difference between the spirometry of obstructive vs. restrictive lung diseases?

Obstructive: asthma, COPD, brionchiolitis
FEV1 = markedly reduced
FVC = relatively unchanged (Except in severe)
FER = Reduced

Restrictive: Idiopathic lung diseases
FEV1 = relatively normal (can be decreased)
FVC = markedly reduced
FER = often remains normal


What diseases encompass obstructive lung diseases? What are the majority of them caused by?

Chronic Bronchitis
Small airways disease


What is chronic obstructive airways disease? COPD?

Often a combination of emphysema, chronic bronchitis and small airways disease.


What are the three types of asthma, and the differences between them?

1. Atopic: IgE mediated to specific allergens
2. Non-atopic: Not IgE mediated to non-specific allergens
3. Other: Not IgE to specific triggers


What are the two temporal responses in asthma? Describe them?

Acute/Immediate: mast cell degranulation: histamine and CLT responses: oedema, bronchospasms and increased mucous production.
Late Response: Epithelial damage and Chemotaxis (eosnophil and cytokine-macrophages) leading to ongoing inflammation.


What are the complications of asthma?

Short term: death, atelectasis (partial collapse or poor lung inflation), spontaneous pneumothorax rare)

Chronic: Airway remodelling (fibrosis and obstruction), chronc hypoxia leading to pulmonary hypertension and cor pulmonale (not very common in asthma)


What is meant by the term status asthmaticus?

Acute, severe asthma that is not responsive to bronchoilators


What is emphysema?

An abnormal, permanent enlargement of air spaces distal to the terminal bronchiole as a result of destruction of the alveoloar wall WITHOUT FIBROSIS


What are the different types of emphysema?

Distal acinar


Describe centriacinar (centrolobar) emphesyma

The most common
Heavily smoking related: smoking causes imbalances of proteases to anti-proteases leading to a net destruction of functional tissue.
An inflammatory response ensues leading to more proteases
Toxins from smoking also directly damage mesenchymal basal (stem) cells leading to overall impaired wound healing ability


What is the macroscopic and microscopic appearances of centriacinar emphysema?

Macroscopically: Formation of big, bubbly airspaces in the lung tissue
Microscopically: loss of alveolar structure (less tissue, more space)


What does emphysema lead to in terms of the mechanics of the lungs?

Causes AIRWAY OBSTRUCTION due to the destruction of elastin and reticulin fibres (loss of ELASTIC RECOIL) in the airways.
Increased risk of COLLAPSE and especially in expiration


What are complications as a result of emphysema?

Hypoxia due to airflow obstruction and loss of diffusion capacity
Pulmonary hypertension leading to cor pulmonale


What are the other forms of "ephysema"

Not actually emphysema but classified so because they cause similar respiratory issues:
- Bullous (large cyst formations, prone to rupture)
- Compensatory (increased air capacity of a portion of a lung because another portion has been dysfunctional - distention without destruction)
- Interstitial (air escapes into the soft tissues)


What is chronic bronchitis?

A persistent cough with productive sputum for at least 3 months in 2 consecutive years

It is a chronic inflammation of the LARGER airways that is almost always caused cigarette smoking with increased mucous production and scarring and narrowing.


What is chronic bronchitis characterised by?

Excessive mucous by hypertrophy of mucous secreting glands
calculated by the RIED index of > 0.4 um and increased number of goblet cells


What is the Ried index?

The ratio between the thickness of the glandular layer vs. the distance between basement membrane and the cartilage. The normal value is 0.4 um


Does fibrosis occur in chronic bronchitis?

Yes, it is characterised by peribronchial fibrosis in the small airways


What are some complications of chronic bronchitis?

Superimposed infective exacerbations (chronic infections usually caused by respiratory pathogens - a major cause of hospital admissions)
Hypoxia, pulmonary hypertension, cor pulmonale
Squamous metaplasia, squamous dysplasia, premalignancy


What kind of metaplasia/dysplasia that occurs in chronic bronchitis?

Tall columnar ciliated cells undergoes metaplasia under stress and trauma causing a change to squamous epithelium (tough, resistant phenotype).

An accumulation of mutations: the cells become atypical (morphologically different) = dysplasia which can eventually lead to squamous cell carcinoma


What is chronic obstructive pulmonary disease (COPD)?

> 90% of cases caused by cigarette smoking
Encompasses: emphysema, chronic bronchitis, small airways disease (with some reversible bronchospasm)


What is COPD characterised by?

A slow progression with superimposed infective exacerbations - bacterial bronchitis and increased bronchospasms


Is the mix for the diseases of chronic bronchitis and emphysema even in COPD?

Not necessarily. Different individuals have different profiles for disease.


What is the difference between Bronchitis vs. Emphysema dominated COPD

Patients tend to tolerate hypoxia better so don't tend to breathe deeply. Become cyanosed and develop cor pulmonale and oedema

Seem intolerant to hypoxia so breath rapidly and deeply and as a result cyanosed


What is smoking a high risk factor in respiration?

Respiratory bronchiolitis-associated with lug disease (RBILD)
Exacerbation of asthma
Lung cancer
Acute bronchitis


How does smoking predispose pulmonary infections?

Inhibition of the muco-ciliary escalator
Increased mucous
Inhibition of leukocyte function
Direct damage to the epithelial layer


What is bronchiectasis?

An IRREVERSIBLE, abnormal dilation of the bronchi/bronchioles

Usually due to a severe or recurrent infection with or without obstruction with the loss of surrounding elastic tissue


What are some causes of bronchiectasis?

Necrotising infections, obstruction and infection, cystic fibrosis, cilia disorders, non-infectious inflammatory disorders (connective tissue disease, graft vs. host disease, allergic bronchopulmonary aspergiliosis)


What is the macroscopic appearance of bronchiectasis?

Ribbed appearance of dilations, large 'holes' in the lungs


What is the clinical course of bronchiectasis?

Severe cough with copious amounts of foul smelling sputum
Episodic Fever
SOB and cyanosis
Cor Pulmonale
Metastatic Infection (brain) Ie. systemic infections


What is restrictive (interstitial) lung disease?

A group of diverse conditions
Chronic, diffuse, non-infectious
Have a restrictive spirometry
Characterised by inflammation and fibrosis of inter-alveolar septa (interstitium)


Prognosis varies widely with different diagnoses. What are the different diagnoses for restrictive lung disease?

Fibrosis - idiopathic pulmonary fibrosis, non-specific interstitial pneumonia, CT diseases, drug reactions, pneumoconiosis
Granulomatous - sarcoidosis, hypersensitivity pneumonitis
Smoking related
Other: pulmonary alveolary proteinosis


What is idiopathic pulmonary fibrosis?

Has an unknown cause
Usual interstitial pneumonitis (histological) with interstitial inflammation and fibrosis at varying stages of development .. Ie. fibrosis at different stages
An inevitable progression to end stage lung disease with a mean survival of 3 years