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Medical Studies 1 > 7.1 & 7.2 Pathology, Diagnosis and Management of Restrictive Lung Disease > Flashcards

7.1 & 7.2 Pathology, Diagnosis and Management of Restrictive Lung Disease Flashcards

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USMLE® Step 1 flashcards
1
Q

What percentage of pulmonary syndromes are restrictive? What about obstructive?

A

Restrictive: 20%
Obstructive: 80%

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2
Q

Using a (not very accurate) acronym, explain five causes of restrictive lung disease

A

PAINT
P: Pleural
A: Alveolar
I: Interstitial
N: Neuromuscular
T: Thoracic cage

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3
Q

What are the three overarching groups of extrinsic restrictive lung disease?

A
  • Decreased muscle tone of respiratory pump
  • Chest wall/rib cage deformities
  • Space-occupying
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4
Q

List a type of disease that falls under “loss of respiratory pump muscle tone” restrictive diseases

A

Neuromuscular disease

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5
Q

List two restrictive chest wall/rib cage deformities

A
  • Obesity
  • Scoliosis
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6
Q

List two space-occupying restrictive lung defects

A
  • Pleural effusion
  • Pneumothorax
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7
Q

Provide a brief explanation of intrinsic restrictive lung diseases

A
  • Reduced distensibility of the lungs
  • Compromised expansion
  • Reduced TLC
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8
Q

Is cough more likely in extrinsic or intrinsic lung disease?

A

Intrinsic

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9
Q

What unique conditions may be present in extrinsic vs intrinsic lung disease?

A

Intrinsic: Connective tissue disease
Extrinsic: Neuromuscular

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10
Q

Diffusing capacity in intrinsic vs extrinsic lung disease

A

Extrinsic: may be normal
Intrinsic: Usually reduced

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11
Q

Which type of respiratory failure is more likely to be present in intrinsic vs extrinsic restrictive lung disease?

A

Intrinsic: Type 1
Extrinsic: Type 2

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12
Q

List some of the main diagnostic features of restrictive lung disease

A
  • Cough (with intrinsic)
  • Systemic features (e.g. malaise, joint symptoms, rash with connective tissue disease)
  • Muscle weakness/neurological symptoms (intrinsic OR extrinsic)
  • Exposure/Past history
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13
Q

Spirometry findings of restrictive lung disease

A
  • Reduced FEV1 and FVC; preserved ratio
  • Or: reduced FVC alone; increased ratio
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13
Q

Lung volume test findings during restrictive lung disease

A
  • Always low TLC (and likely low other volumes)
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14
Q

ABG findings in intrinsic vs extrinsic restrictive lung disease

A
  • May be hypoxia (caused by V/Q mismatch and increased pulmonary shunting with intrinsic)
  • Or hypercapnia (more common with extrinsic)
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15
Q

List two conditions that can decrease chest wall compliance

A
  • Obesity
  • Kyphoscoliosis
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16
Q

What does it mean that anatomical dead space increases in idiopathic pulmonary fibrosis?

A

The amount of conducting airway increases relative to lung volumes

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17
Q

What happens to the amount of alveolar dead space present during idiopathic pulmonary fibrosis?

A

It increases

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18
Q

Why does exercise worsen hypoxaemia in patients with idiopathic pulmonary fibrosis?

A
  • Lower capillary transit time
  • Cannot recruit enough lung volume to oxygenate the blood
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19
Q

Why does awake hypercapnia only occur in end-stage restrictive intrinsic lung disease?

A

The respiratory muscles fail due to overwhelming mechanical load from severely reduced compliance

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20
Q

How can neuromuscular and chest wall respiratory disorders cause cor pulmonale?

A
  • Hypoxaemia
  • Pulmonary vasoconstriction -> increased pulmonary vascular resistance
  • Right heart failure
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21
Q

Most common space-occupying lesion leading to restrictive lung disease

A

Pleural effusion

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22
Q

List two gas exchange abnormalities in restrictive lung disease

A
  • Intrapulmonary shunt
  • Alveolar dead space (in heart failure)
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23
Q

Define interstitial lung disease

A

Disease that affects lung interstitium (as opposed to airways)

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24
Q

Summarise ILD pathophysiology

A
  • Inflammation of parenchyma
  • Collagen in interstitium
  • Irreversible pulmonary fibrosis
  • Decreased lung compliance
  • Decreased inspiratory capacity
  • Thickened alveolar septae
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25
Q

List some occupational/inhaled exposures that can cause interstitial lung disease

A
  • Silica
  • Asbestos
  • Beryllium
  • Birds
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26
Q

List some pneumotoxic drugs

A
  • Antibiotics
  • Chemo
  • Antiarrhythmic
  • Statins
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27
Q

What are pneumoconioses?

A

Group of lung diseases caused by inhalation of certain dusts

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28
Q

Is lung fibrosis better or worse than lung inflammation?

A

Worse; irreversible scarring

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29
Q

Define occupational lung disease

A
  • Lung diseases that arise as a result of occupational exposure to dust, fumes, smoke, or other agents
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30
Q

Screening history questions for occupational lung disease

A
  • What kind of work (exactly)
  • Do they think breathing problems are related to work?
  • Do symptoms get better when you are away?
  • Have you been exposed to fumes, gases, or dusts in the past?
31
Q

History questions to ask IF occupational exposure to substances was present

A
  • Type of substance
  • Frequency, quantity duration
  • Route of exposure
  • Protective equipment?
  • Similar symptoms in coworkers?
32
Q

Non occupational exposures that can cause lung disease

A
  • Smoking
  • Pets
  • Home environment
  • Hobbies/recreation
33
Q

What are the two types of work related asthma?

A
  • Occupational asthma
    -Work exacerbated asthma
34
Q

What are the two classifications of occupational asthma? Which is more common?

A
  • Sensitiser-induced (immunological)
  • Irritant-induced (non-immunological;l epithelial asthma)

Sensitiser induced is more common (~90%)

35
Q

How can peak flow measurements be used in diagnosis of occupational asthma? What are the limitations of this?

A
  • If peak flow decreases during work and increases after work, then it is indicative of work related asthma
  • However, this does not differentiate between sensitiser-induced and irritant-induced
36
Q

What percentage of COPD cases are linked to occupational exposures?

A

15%

37
Q

What are the three main types of occupational interstitial lung disease?

A
  • Pneumoconiosis
  • Granulomatous
  • Hypersensitivity pneumonitis
38
Q

What characterises granulomatous lung disease?

A

Granuloma formation

39
Q

What is hypersensitivity pneumonitis? What substances can trigger it?

A
  • Parenchymal inflammation as an allergic reaction to inhaled substances
  • Substances include bacteria, fungi, animal proteins, low molecular weight chemicals
40
Q

Does asbestos-related disease have a long or short delay before onset? How long or short is it?

A

Long. Over 25 years.

41
Q

List some common industries that can lead to asbestos exposure

A
  • Insulation
  • Cement production
  • Dockyard work
  • Railway carriage maintenance
42
Q

What main three types of disease are caused by asbestos exposure?

A
  • Interstitial lung disease
  • Pleural disease
  • Malignancy
43
Q

What are pleural plaques? What are they made of? Do they increase cancer risk?

A
  • Collagen components with a linear, banded, or nodular appearance.
  • There is no accepted correlation between pleural plaques and cancer risk
44
Q

Describe pleural thickening. Under what circumstances can it cause dyspnoea? Under what circumstances could it indicate cancer?

A
  • Fibrosis involving the visceral and parietal pleura
  • May reduce lung volumes and cause dyspnoea
  • If in conjunction with chest wall pain, may indicate malignancy
45
Q

What is pleural mesothelioma?

A

Cancer that forms in the tissue covering your lungs and chest wall

46
Q

Symptoms of benign asbestos-related pleural effusion. Is this always the case?

A
  • Pleuritic chest pain
  • Dyspnoea
  • Chronic cough
  • Fever

However, asymptomatic in a majority of cases

47
Q

Describe benign asbestos pleural effusions. How long before onset of interstitial lung disease do they occur?

A
  • Usually small and unilateral
  • They occur years before the onset of interstitial lung disease
48
Q

Correlation between amount of asbestos exposure and earlier onset of benign pleural effusions

A
  • Largely unclear
  • Earlier onset may be correlated with higher amounts of exposure
49
Q

What is pulmonary asbestosis?

A

Pulmonary fibrosis caused by inhalation of asbestos fibres

50
Q

Does the classification of substances as carcinogens inform treatment at an individual level?

A

No; only at a population level

51
Q

What is the most common form of cancer in never smokers and asbestos exposure patients?

A

Adenocarcinoma

52
Q

What is adenocarcinoma?

A

Cancer in epithelial tissue of glands

53
Q

What type of respiratory failure is more common in interstitial lung disease? Why?

A

Type 1; the respiratory pump can still remove CO2, the problem is at the level of gas exchange and O2 transport inward.

54
Q

What is meant by ground-glass opacity?

A

Areas of hazy greyness that do not obscure underlying structures.

55
Q

What is reticulation on a CT scan?

A

“Like a net”

56
Q

What histological findings on biopsy support a diagnosis of interstitial pneumonia? Will there be more fibrosis or inflammation?

A
  • Identification of tissue at varying stages of fibrosis within the same sample.
  • There will be more fibrosis than inflammation
57
Q

What is the gold standard for IPF diagnosis?

A

Multidisciplinary diagnosis

58
Q

Criteria for IPF diagnosis

A
  • Common clinical features and exclusion of other causes
  • Histological pattern of UIP
59
Q

A patient has inhaled a gas/particulate that could damage their lungs. Generally, which zone(s) of the lungs are most likely to be damaged as a result?

A

The upper lung zones

60
Q

List some clinical findings of ILD on examination

A
  • Clubbing
  • Cough
  • Fine inspiratory crackles
61
Q

What could high attenuation on lung CT indicate?

A

Consolidation

62
Q

What could low lung attenuation on lung CT indicate?

A

Emphysema

63
Q

List some types of conditions that favour the upper lung zones

A
  • Pneumoconiosis
  • Smoking-related diseases
  • Hypersensitivity pneumonitis
64
Q

List some conditions that favour the lower zones of the lungs

A
  • Pulmonary oedema
  • Aspiration
  • Usual interstitial pneumonia
65
Q

Consolidation vs ground-glass-opacity

A

Consolidation: Cannot see underlying vessels
Gound-glass: can see underlying vessels

66
Q

Describe treatment of interstitial lung disease

A
  • Remove irritant
  • Treat exacerbations
  • Steroids may help reverse inflammation
  • Lung transplantation
  • Medico-legal aspects
  • Ongoing monitoring
67
Q

What is lung consolidation?

A

It indicates that the normal, air-filled spaces of the lung are filled with the products of disease

68
Q

List five broad categories of restrictive lung disease

A
  • Occupational
  • Autoimmune
  • Treatment related
  • Sarcoidosis
  • Idiopathic
69
Q

Asbestosis pathology

A
  • Fibrosis of lower lung lobes
  • Fibrosis/collagen plaques of surrounding pleura
70
Q

Asbestosis pathophysiology

A
  • Asbestos fibres have a toxic effect on cells
  • Release of inflammatory mediators leads to fibrosis
71
Q

Mesothelioma pathology

A
  • Starts as small nodules (typically on parietal pleura)
  • Over time, grows larger and joins together, forming larger tumour
  • Can invade surrounding structures, such as the chest well
72
Q

Treatment of neuromuscular breathing disease

A
  • Respiratory therapy
  • Home education
  • Physiotherapist for exercise
73
Q

Does fibrosis commonly result from chronic or acute inflammation?

A

Chronic

74
Q

What are the signs and symptoms that result from inflammation of the lung interstitium?

A

Interstitial fibrosis -> decreased compliance, and thickened alveolar membrane (leading to impaired diffusion)

Signs:
- End-inspiratory fine crackles

Symptoms:
- Dyspnoea

75
Q

Symptoms caused by inflammation of bronchi

A
  • Excess mucus production
  • Productive cough
  • Dyspnoea
76
Q
A

Medical Studies 1 (90 decks)

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