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CARDIO & RESPIRATORY phase 1b > 1b Restrictive Lung Disease > Flashcards

1b Restrictive Lung Disease Flashcards

1
Q

What is a restrictive lung disease?

A

When the expansion of the lungs is restricted

Lung volumes are small

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2
Q

What are the two types of restrictive lung disease?

A

Intrinsic Lung Disease
Extrinsic Lung Disease

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3
Q

What does intrinsic lung disease involve?

A

Alterations to the lung parenchyma = interstitial lung disease

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4
Q

What does extrinsic lung disease involve?

A

Compression of the lungs / limiting expansion
- pleural
- chest wall
- neuromuscular = decreases the ability of the respiratory muscles to inflate / deflate the lungs

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5
Q

What is the lung parenchyma?

A

The alveolar regions of the lung

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6
Q

What are the four cellular components of the lung parenchyma?

A
  1. Alveolar type 1 epithelial cell - they provide the surface area for gas exchange to occur on, most common type
  2. Alveolar type 2 epithelial cell - they secrete sufactat and also are able to convert into alveolar type 1 cells
  3. Fibroblasts - secrete ECM like type 2 collagen
  4. Alveolar macrophages - inject pathogens and also release surfactant
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7
Q

What is the interstitial space?

A

The space between the alveolar epithelium and the capillary endothelium - provides structural support to the lungs and very thin to facilitate gas exchange

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8
Q

What does the interstitial space contain?

A

Lymphatic vessels, occasional fibroblasts and ECM

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9
Q

What is the purpose of alveolar type 1 epithelial cells?

A

Gas exchange surface

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10
Q

What is the purpose of alveolar type 2 epithelial cells?

A

They release a surfactant to reduce surface tension and are stem cells needed for repair

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11
Q

What do fibroblasts do?

A

Produce ECM like collage type 1

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12
Q

Which alveolar epithelial cell can differentiate?

A

Type 2 -> into type 1

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13
Q

What is the purpose of alveolar macrophages?

A

Phagocytose foreign material, and release surfactantx

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14
Q

Which immune cell are closely associated with the lung epithelium?

A

Alveolar macrophages

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15
Q

What characterises interstitial lung disease?

A

inflammation or fibrosis in the interstitial space

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16
Q

What are some causes/ examples of ILD?

A

Idiopathic
Autoimmune related
Exposure related
With syst or airspace filling
Sarcoidosis
Eosinophilic pneumonia

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17
Q

What is the common history of a patient with Interstitial Lung Disease?

A

Progressive Breathless
Non-productive cough
Limitations in exercise tolerance
Occupational history

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18
Q

What are the clinical examinations seen in patients with interstitial lung disease?

A

Low O2 saturations
Fine bilateral inspiratory crackle
Digital clubbing

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19
Q

What is the 6 minute walk test?

A

make patients walk for 6 minutes and if they desaturate below 88% then this is a good marker of interstital lung disease

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20
Q

What are the main investigations into ILD?

A

High Resolution CT - look at the pattern of disease

Blood tests e.g. anti-nuclear antibody (ANA), rheumatoid factor (RhF), anti-citrullinated peptide (CCP)

Pulmonary function tests

6 minute walk test

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21
Q

What are the two invasive tests which might be done for a patient with ILD?

A
  1. Bronchoalveolar lavage - flood lungs with saline and see how much can / is removed
  2. Surgical Lung Biospy
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22
Q

Why does the compliance of the lung decrease in ILD?

A

Scarring makes the lung stiff

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23
Q

What happens to Forced Vital Capacity in ILD?

A

decreases

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24
Q

What happens to the diffusing capacity of lung for CO in ILD?

A

Decreases

Diffusion capacity assesses how well a tracer gas in inspired air can cross from the air into the blood.

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25
Q

What happens to the FEV1/FVC ratio in ILD?

A

Normal or increased

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26
Q

What type of pattern is seen on a HRCT in Interstitial pneumonia?

A

Honey comb

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27
Q

What pattern is seen on a HRCT in organising pneumonia?

A

Consolidation = dense white

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28
Q

What are the general principles of ILD management in Early Disease?

A

Pharmacological treatments
Patient education
Vaccination
Smoking cessation
Treatment of co-morbidities
Pulmonary rehabilitation

29
Q

What are the general principles of ILD management in late disease?

A

Supplemental O2
Lung transplant
Palliative Care

30
Q

What is idiopathic pulmonary fibrosis?

A

Progressive, scarring lung disease or unknown cause

31
Q

Which gender is idiopathic pulmonary fibrosis more common in?

A

Men

32
Q

What is the average decline in FVC?

A

150-200mls/year

33
Q

What is the prognosis of Idiopathic pulmonary fibrosis?

A

poor, even lower survival rate than many cancers

34
Q

What are the three predisposing factors of IPF?

A

Genetic susceptibility (MUC5B, DSP)
Environmental triggers
Cellular Ageing

35
Q

What is the mechanism of IPF?

A

Injury to the endothelial lining
Aberrant fibroblast activity
Excess accumulation of ECM
Results in remodelling and honeycomb cyst formation

36
Q

What effect does remodelling have on the lung?

A

Honey comb cysts - make the lung less efficient at gas exchange

37
Q

What event initiates IPF?

A

Alveolar epithelial injury

38
Q

Describe the histopathological changes which are seen with IPF?

A

Microscopic honeycomb cysts
Fibroblastic foci = proliferating myofibroblasts

39
Q

What are the key features of interstitial lung disease?

A

fine bibasal inspiratory crackles and finger clubbing, with low oxygen saturations

40
Q

What used to be the conventional therapy for IPF?

A

Immunosuppression

41
Q

What is the most useful treatment for IPF now?

A

Anti-fibrotics - THEY SLOW PROGRESSION BUT DO MOT CURE

42
Q

What are two anti-fibrotics?

A

Nintedanib (tyrosine kinase inhibitor) and Pirfenidone (a pyridine compound)

43
Q

What us hypersensitivity pneumonitis?

A

ILD caused by immune mediated response in susceptibile and sensitized individuals to inhaled environmental antigens

44
Q

What is involved in hypersensivity pneumonitis?

A

Involves small airways and parenchyma

45
Q

What are the two different types of hypersensitivity pneumonitis?

A

Acute HP and Chronic HP

46
Q

What is the difference between Acute and Chronic HP?

A

Acute = intermittent, high level exposure with abrupt symptom onset

Chronic = long term, low level exposure, and non-fibrotic = purely inflammatory
fibrotic = associated with higher mortality

47
Q

What drives the pathophysiology of hypersensitivity pneumonitis?

A

Immunological dysregulation - involves the innate and adaptive immune system

48
Q

What mediates the inflammatory response seen in HP?

A

T-Helper cells and antigen-specific IgG antibodies

49
Q

What does the immunological dysregulation of HP result in the formation of?

A

Accumulation of lymphocytes and formation of granulomas

50
Q

Why is the exposure history so important in the diagnosis of HP?

A

Antigen is not identified in about half the cases

51
Q

What can be found in a diagnostic work up of HP?

A

Inspiratory ‘squeaks’ on auscultation - caused by the coexisting bronchiolitis

Specific circulating IgG antibodies to potential antigens

HRCT

52
Q

What bronchoalveolar lavage lymphocyte count is a good indicator of HP?

A

> 30%

53
Q

What does bronchiolocentric inflammation show on a HRCT

A

Ground glass nodules

54
Q

What is the treatment of HP?

A
  • Complete removal of the antigen
  • Use corticosteriods
  • Immunsuppresants such as MMF and azathioprine
  • if progressive and fibrotic, use Nintedanib = anti-fibrotic
55
Q

What is SSc associated ILD?

A

An autoimmune connective tissue disease characterised by dysregulation and progressive fibrosis that affects the skin, with variable internal organ involvment

56
Q

Who does SSc ILD effect?

A

Young, middle aged women

57
Q

What are the clinical features of SSc\?

A

Raynaud’s
Sclerodactyly
Abnormal nail fold capillaroscopy
Digital Ulcer
Telengectasias - broken small blood vessels near the surface of the skin

58
Q

What is the difference between limited cutaneous SSc and Diffuse SSc?

A

Limited = only skin involvement up to elbows, diffuse = involves the whole body

59
Q

What is different about SSc compared to other ILD’s?

A

SSc has a vascular component

60
Q

What HRCT is seen in SSc-ILD?

A

Non-specific interstitial pneumonia (NSIP) pattern is the most common pattern

61
Q

What determines which treatment is given for SSc-ILD?

A

Determined by disease extent on HRCT and lung function trajectory (monitor every 3 – 6 months)

62
Q

What are the management options for SSc-ILD?

A

corticosteroid use is controversial and risk of renal crisis with high doses (>10mg/day)

Immunosuppressives – cyclophosphamide, mycophenolate mofetil (MMF)1

Progressive fibrotic phenotype– Nintedanib (antifibrotic)2

63
Q

What is the difference in FEV1/FVC and the ratio. between restrictive and obstructive conditions?

A

restrictive: FEV1 and FVC are both low, therefore the ratio is high

obstructive = even lower FEV1, so ratio is low

64
Q

What anti-fibrotic is used to treat idiopathic pulmonary fibrosis?

A

pirfenidone

65
Q

what are common exam question indications of HP?

A

Bird droppings, and rat faeces

66
Q

What are the three CT scan things which are associated with HP?

A

Chronic honeycombing, ground glass and traction bronchiectasis

67
Q

What are the auto antibodies associated with Limited Cutaenous SSc?

A

Anti-Centromere

68
Q

What is the auto-antibody associated with Diffuse Cutaenous SSc?

A

Anti-Scl70

69
Q

How to differentiate between Limited and Diffuse SSc?

A

Limited = Raynauds
Diffuse = fibrosis

CARDIO & RESPIRATORY phase 1b (14 decks)

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