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BRS CARDIO & RESPIRATORY > 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
  2. Alveolar type 2 epithelial cell
  3. Fibroblasts
  4. Alveolar macrophages
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7
Q

What is the interstitial space?

A

The space between the alveolar epithelium and the capillary endothelium - filled with collagen and matrix which restricts the diffusion of oxygen into the lungs

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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 collagen 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 surfactant

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14
Q

Why is the interstitial space thin?

A

In order to facilitate gas exhange

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15
Q

Which immune cell are closely associated with the lung epithelium?

A

Alveolar macrophages

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16
Q

What characterises interstitial lung disease?

A

inflammation or fibrosis in the interstitial space

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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

(+/-features of connective tissue disease- skin, joints, muscles)

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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 is the mainstay investigation into ILD?

A

High Resolution CT - look at the pattern of disease

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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 Biopsy
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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 IDL?

A

Decreases

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25
Q

What happens to the FEV1/FVC ratio in ILD?

A

Normal or increased

26
Q

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

A

Honey comb

27
Q

What pattern is seen on a HRCT in organising pneumonia?

A

Consolidation = dense white

28
Q

What are the general principles of ILD management in Early Disease (6)?

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 of unknown cause

31
Q

Which gender is idiopathic pulmonary fibrosis more common in?

A

Men

32
Q

What is the prognosis of Idiopathic pulmonary fibrosis?

A

poor, even lower survival rate than many cancers

33
Q

What are the three predisposing factors of IPF?

A

Genetic susceptibility- MUC5B + DSP
Environmental triggers- smoke, viruses, pollutants dust
Cellular Ageing- telomere attrition, senescence

34
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

35
Q

What effect does remodelling have on the lung?

A

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

36
Q

What event initiates IPF?

A

Alveolar epithelial injury

37
Q

Describe the histopathological changes which are seen with IPF?

A

Microscopic honeycomb cysts
Fibroblastic foci = proliferating myofibroblasts

38
Q

Where does IPF originate from?

A

The base / periphery of the lung and moves inwards

39
Q

What used to be the conventional therapy for IPF?

A

Immunosuppression

40
Q

What is the most useful treatment for IPF now?

A

Anti-fibrotics

41
Q

What are two anti-fibrotics?

A

Nintedanib and Pirfenidone

42
Q

What is hypersensitivity pneumonitis?

A

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

43
Q

What is involved in hypersensivity pneumonitis?

A

Involves small airways and parenchyma

44
Q

What are the two different types of hypersensitivity pneumonitis?

A

Acute HP and Chronic HP

45
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

46
Q

What drives the pathophysiology of hypersensitivity pneumonitis?

A

Immunological dysregulation - involves the innate and adaptive immune system

47
Q

What mediates the inflammatory response seen in HP?

A

T-Helper cells and antigen-specific IgG antibodies

48
Q

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

A

Accumulation of lymphocytes and formation of granulomas

49
Q

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

A

Antigen is not identified in about half the cases

50
Q

What can be heard in patients with HP?

A

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

51
Q

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

A

> 30%

52
Q

What does bronchiolocentric inflammation show on a HRCT

A

Ground glass nodules

53
Q

What is the treatment of HP?

A
  • Complete removal of the antigen
  • Use corticosteriods
  • Immunsuppresants
  • if progressive and fibrotic, use Nintedanib = anti-fibrotic
54
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

55
Q

Who does SSc ILD effect?

A

Young, middle aged women

56
Q

What are the clinical features of SSc?

A

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

57
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

58
Q

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

A

SSc has a vascular component

59
Q

What HRCT is seen in SSc-ILD?

A

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

60
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)

61
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)

BRS CARDIO & RESPIRATORY (26 decks)

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