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Stage 2: Supporting Life > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

What is the characteristic feature of restrictive lung disease?

A

Reduced lung volume

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2
Q

What areas does intrinsic restrictive lung disease involve?

A

Lung parenchyma

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3
Q

What areas does extrinsic restrictive lung disease involve?

A
  • Pleura
  • Chest wall (incl. obesity)
  • Neuromuscular diseases (respiratory pump)
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4
Q

What do lung function tests show in restrictive lung disease?

A

1) Reduced vital capacity
2) Preserved airflow (FEV1)
3) Increased FEV1/FVC
4) Gas transfer → reduced in lung parenchymal disease but preserved in other causes of RLD e.g. motor neurone disease

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5
Q

What should expiration be?

A

Effort independent

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6
Q

What is an example of a chest wall disease that causes RLD?

A

Kyphoscoliosis

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7
Q

What happens in kyphoscoliosis?

A

1) Spine is curved so the shape of the chest is wrong

2) This prevents lung expansion bc can’t expand the lungs as much as are supposed to

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8
Q

How can kyphoscoliosis lead to type 2 respiratory failure?

A

1) Progressively as the lungs are getting more and more squashed, patients can go into type 2 respiratory failure
2) Therefore they are no longer able to compensate → during the day they can breathe normally but when sleeping CO2 rises and in the morning they normally compensate but this eventually goes

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9
Q

What are causes of diffuse pleural thickening leading to RLD?

A

Infection, asbestos

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10
Q

What happens in diffuse pleural thickening?

A
  • The lining of the lung (pleura) becomes inflamed and solid
  • This can happen bc of asbestos exposure years later or infection making pleura thickened and hardened or haemothoraces
  • This prevents lung expansion and restricts how you breathe
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11
Q

What are causes of extrinsic restriction?

A

1) Pleural diseases
2) Chest wall disease/deformity
3) Obesity → increased amount on chest, unable to inflate chest (restriction)
4) Neuromuscular

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12
Q

What is the effect of having obesity-associated hypoventilation?

A

Survival gets progressively worse as don’t breathe as well as you should overnight

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13
Q

What are features of intrinsic restriction (ILD)?

A

1) ‘Reduced lung volume’
2) Increased elastic recoil of lung
3) Hypoxia due to VQ mismatch - lung is damaged → hypoxia

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14
Q

What is interstitial lung disease (ILD)?

A

Fibrotic lung disease causing thickening of lung tissue

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15
Q

What is the main ILD?

A

Idiopathic pulmonary fibrosis

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16
Q

What are examples of associations in ILD of known association?

A

1) CTD (chemotherapy drug)
2) Medication
3) Exposure

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17
Q

What are other names for ILD?

A

1) Pulmonary fibrosis

2) Lung scarring

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18
Q

What are key features of ILD?

A

1) Interstitial inflammation → fibrosis
2) Impaired gas exchange → VQ mismatch
3) Symptoms → SOB/cough
4) Abnormal CXR (not always) → CT scan better
5) Abnormal lung function → restriction, reduced lung volumes and gas transfer factor decreases

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19
Q

What are symptoms of ILD?

A
  • Breathlessness
  • Dry cough
  • Wheeze
  • Chest pain
  • Haemoptysis
  • Fever, myalgia → if linked to CT disease
  • Weight loss
  • Weakness
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20
Q

What might you see on general inspection of someone with ILD?

A
  • Pt might be underweight
  • Might have chest/spine deformity e.g. in ankylosing spondylitis
  • Rash → in CT disease
  • Tachypnoea
  • Clubbing
  • Accessory muscle use (if later stage)
  • Cyanosis (also later stage)
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21
Q

What might you see in a respiratory examination of someone with ILD?

A
  • Tracheal deviation
  • Reduced chest expansion
  • Altered percussion note
  • Inspiratory crepitations (crackles)
  • Reduced/absent breath soudns
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22
Q

Why might fibrosis not always be uniform/bilateral?

A

If patients sleep on one side more likely to get fibrosis on that side e.g. if have severe reflux acid might tip onto that side

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23
Q

What might you see in a cardiovascular examination on someone with ILD?

A
  • Displaced apex beat
  • Signs of secondary pulmonary hypertension (hypoxic)
  • Signs of cor pulmonale (hypoxic)
  • Loud P2, fluid overload
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24
Q

What disease is the pathology of idiopathic similar to?

A

Interstitial pneumonia

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25
Q

What are the main causes of clubbing and crackles?

A
  • Idiopathic pulmonary fibrosis
  • Bronchiectasis
  • Cystic fibrosis (bc in CF get bronchiectasis)
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26
Q

What questions might you ask someone with pulmonary fibrosis?

A
  • Occupation
  • Drugs
  • Previous infections causing damage e.g. TB, whooping cough, pneumonia
  • Hobbies/pets (birds) → hypersensitivity pneumonitis (allergy)
  • Smoking → respiratory bronchiolitis ILD (RBILD), gets better if stop smoking
  • Family history
  • Systemic enquiry
  • What bedding/pillows made of? e.g. allergy to bird feathers (avian precipitins)
  • Radiotherapy for cancer e.g. breast directed to chest → beam can damage lung
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27
Q

Why might you ask someone with PF about their occupation?

A
  • Asbestosis → ship building, pipe work, electricians, building work
  • Farmers lung → chemical exposure, compost (allergy)
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28
Q

Why might you ask someone with PF about their drug history?

A
  • Amiodarone (anti-arrhythmic)
  • Nitrofurantoin → still given prophylactically for recurrent UTIs
  • Drugs only cause the reaction in some people (so also other drugs)
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29
Q

Why might you do a systemic enquiry on someone with PF?

A

Looking for CT disease

  • Eyes
  • Pain/swelling of joints incl. fluctuating joint aches
  • Rash
  • Weakness
  • Raynaud’s
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30
Q

What bloods would you do for someone with ILD?

A

FBC, U+E, autoantibodies (ANA and ENA), rheumatoid factor

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31
Q

Why would you do an FBC on someone with ILD?

A

To test renal function bc patients get vasculitis and with this renal involvement
- e.g. in goodpasture syndrome (GPS), ANCA positive vasculitis, granulomatosis with polyangiitis (GPA)

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32
Q

What autoantibodies would you test for in someone with ILD?

A

RF, CCP antibody, myositis antibodies, complement factors

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33
Q

On what are ABGs taken?

A

Air

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34
Q

What is type 1 respiratory failure?

A

High pO2, normal pH and pCO2

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35
Q

What is a high FEV1/FVC ratio?

A

> 75% e.g. 87%

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36
Q

What might you see on a CT of someone with ILD?

A
  • Basal, bilateral sub pleural reticular changes
  • Honeycombing → holes punched out, can’t treat, just need to stop progression (also seen in emphysema)
  • Traction bronchiectasis
37
Q

What happens in traction bronchiectasis?

A
  • Airways are bigger than they should be
  • Lungs are becoming smaller (shrinking is bad prognostic sign), pulling the airways
  • Don’t bring up a lot of phlegm unlike patients with normal bronchiectasis
38
Q

What are risk factors for idiopathic pulmonary fibrosis?

A
  • Smoking
  • Metal/wood dust
  • Genetic (familial)
39
Q

Why is idiopathic pulmonary fibrosis a problem with emphysema?

A

Bc if have a lot of emphysema as well, lung volumes are quite high and not reduced where you need them to be for the medication

40
Q

Is IPF more common in men or women?

A

Men

41
Q

What is the mean age of IPF?

A

66

42
Q

What is the pathogenesis of usual interstitial pneumonia?

A
  • Aetiology unknown
  • Proliferation of mesenchymal cells, collagen deposition, fibroblastic foci
  • Minimal inflammation, fibrosis predominates
  • Abnormal epithelial repair to unknown stimulus (hypothesis)
  • IFN, TNF, TGF, oxidants important in pathogenesis
43
Q

What investigations do you do in someone with IPF?

A
  • Bloods → FBC, AA, RhF
  • CXR/HRCT
  • Lung function test
  • Oxygen saturation (blood gases)
  • Lung biopsy in some cases but if they have a complication with these lungs won’t end well
  • Bronchoalveolar lavage
44
Q

Why do you do a lung function test on someone with IPF?

A

To know if they are hypoxic to give them supportive treatment

45
Q

Describe bronchoalveolar lavage

A
  • Looking for infection (look at inflammatory profile)
  • Put some fluid in a send it off to cytology who report on the number of immune cells
  • Get lots of lymphocytes in allergic disease e.g. hypersensitive pneumonitis
46
Q

What do you do every time you are monitoring someone with IPF?

A

Lung function and ask how they feel

47
Q

How do you diagnose IPF?

A

CT ± lung biopsy

48
Q

How do you assess severity of IPF?

A

Lung function - VC, TLCO

49
Q

How do you work out the prognosis of someone with IPD?

A

Exercise test/serial VC

50
Q

What is the aim of IPF treatment?

A

To reduce disease progression

51
Q

What two drugs are used to reduce progression of IPF?

A
  • Pirfenidone (antifibrotic)

- Nintedanib (triple tyrosine kinase inhibitor)

52
Q

What are the effects of pirfenidone and nintedanib?

A
  • Oral
  • Slows disease progression (FVC)
  • Reduces mortality
53
Q

What are side effects of drugs used to treat IPF?

A

GI, diarrhoea

54
Q

What is used to support patients with IPF when they are hypoxic?

A

Oxygen

55
Q

What are other treatments for people with IPF?

A

Pulmonary rehabilitation
Palliative care
Lung transplant

56
Q

Describe pulmonary rehabilitation

A
  • Works v well
  • Maybe bc coaching lungs into accepting breathlessness better
  • Getting people into more psychological frame of mind in how to manage this
  • Training respiratory muscles
57
Q

Why do patients with IPF not really get lung transplants?

A

Because lung thickening makes lungs harder to transplant

58
Q

What is the median survival for IPF?

A

3 years esp. in idiopathic group (poor, not much better than lung cancer)

59
Q

What does UIP stand for?

A

Usual interstitial pneumonia

60
Q

What does NSIP stand for?

A

Non specific interstitial pneumonia

61
Q

What is the main cause of death in IPF patients?

A

Respiratory failure

62
Q

What are other causes of death in IPF patients?

A
  • Heart failure
  • PE
  • Pneumonia
  • Lung cancer (high incidence)
63
Q

Describe differences between NSIP (IIP) and UIP

A
  • CT → see ground glass opacities and fibrosis - little holes but also patches with more normal lung around
  • More inflammatory
  • Treatment responses better
  • Prognosis better
64
Q

What is the drug treatment for NSIP/DIP/AIP/COPD?

A

Steroids+

65
Q

What is the treatment for HP (hypersensitivity pneumonitis)?

A

Steroids

66
Q

What is the treatment for connective tissue disease?

A

Immunosuppressants e.g. rituximab (wipes out B cells)

67
Q

What are systemic effects of ILD?

A
  • Systemic sclerosis (red dots on face?)
  • Dermatomyositis (rash under eyes)
  • Changes in knuckles → papules, machinist hands, rough
  • SLE
  • Rheumatoid arthritis → shouldn’t see this now bc patients with suspected diagnosis will be seen within 4-6 weeks and will v quickly be put on DMARDs (disease modifying anti-rheumatic drugs)
  • Ankylosing spondylitis
68
Q

What is sarcoidosis?

A

A multi-system disease that can affect any organ (genetic in < 5% of cases)

69
Q

What organs are most commonly affected by sarcoidosis?

A

Lungs, skin, eyes (uveitis), kidney, heart, liver

70
Q

What do most people with sarcoidosis die of?

A

Respiratory failure bc lungs are the most common organ affected

71
Q

What happens when sarcoid affects the heart?

A
  • Severe disease
  • Affects electrical systems leading to arrhythmias, heart block, ventricular tachycardia
  • Typical symptos of pre-syncopal episodes, syncopal episodes, breathlessness
72
Q

What happens when sarcoid affects the neuro system (CNS or PNS)?

A

Facial nerve palsy, cognitive features

73
Q

Why is prognosis of sarcoid better than fibrosis?

A

Bc lots of people have mild disease

74
Q

What are causes of sarcoidosis?

A

1) Unknown
2) Inhaled → infection, other antigen
3) Inflammatory disease (T cell)

75
Q

What is a hallmark lesion of sarcoidosis on a biopsy?

A

Granuloma (but are other causes e.g. TB which hasn’t necrotised yet)

76
Q

What is the acute presentation of someone with sarcoidosis?

A
  • Loefgren’s syndrome
  • Bilateral hilar lymphadenopathy on CXR
  • Erythema nodosum (rash over legs, raised, painful papules)
  • Arthralgia
  • Good prognosis - no problems usually, hilar lymphadenopathy may or may not be clear
  • Don’t usually need a biopsy
77
Q

What is the chronic presentation of someone with sarcoidosis?

A
  • Can be self limiting
  • Can also relapse
  • Can become progressive
  • Can be v difficult to treat bc lung keeps on getting eroded and destroyed
78
Q

What are investigations that you should do for sarcoidosis?

A
  • CXR (scadding) but CT is better
  • Serum ACE
  • Calcium
  • Liver FT (sarcoid affects liver)
  • Renal
  • FBC
  • Biopsy
  • Lung function test
  • CT scan chest
  • Heart → baseline 24h ECG and cardiac MRI/PET to look for scarring and cardiac inflammation
79
Q

Describe the serum ACE test for sarcoidosis

A
  • Used as test but not definitively diagnostic as can be elevated for other reasons and can be normal
  • If have proven sarcoid and it goes down in treatment, it can be a useful marker
80
Q

Describe calcium test for sarcoidosis

A
  • Sarcoid/granulomas interfere with calcium metabolism so patients quite often present with hypercalcaemia
  • Bigger risk in summer when patients are exposed to the sun
  • Can affect kidneys
81
Q

Why do you do an FBC for someone with sarcoid?

A

Some patients can have bone marrow failure

82
Q

Describe biopsy for sarcoidosis

A
  • Could be something else
  • EBUS (endobronchial US to get mediastinal lymph node biopsy)
  • Skin lesion
  • Lymph node
83
Q

What is included in treatment for sarcoidosis?

A

Immunosuppression principles (QoL and threatened organ function)

  • Steroids
  • Methotrexate and/or hydroxychloroquine if need second line treatment
  • Anti-TNF
  • Mycophenolate, azathioprine
84
Q

What happens for many patients with sarcoid?

A

They don’t require any treatment, just observation/monitoring

85
Q

What are the outcomes of sarcoidosis?

A
  • Good life expectancy even in progressive disease → however more indirect mortality not captured e.g. effects of steroids or heart attack
  • Pulmonary hypertension → poor prognosis
86
Q

What is a mesothelioma?

A

A pleural tumour

87
Q

What happens to pleura in mesothelioma?

A

Pleura is infiltrated, thick and hardened

88
Q

What can you see on a CXR in pulmonary fibrosis?

A
  • Reticular nodular shadowing seen in pulmonary fibrosis
  • This is where CXR can be useful but if getting CXR at this stage in condition it is useless → something has gone wrong if this is the first CXR you see

Stage 2: Supporting Life (30 decks)

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