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M103 T4 > M103 T4 L13 > Flashcards

M103 T4 L13 Flashcards

1
Q

What are the two types of xray shadowing?

A

alveolar

interstitial

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2
Q

What four structures are included under the interstitium?

A

alveolar endothelium
capillary endothelium
basement membrane
connective tissue

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3
Q

Which three things can thicken the interstitium in disease?

A

Fluid
Cells
Fibrosis

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4
Q

What is lung fibrosis thought to be caused by?

A

aberrant wound healing
the lung could be exposed to an injury
the wound healing mechanism goes into overdrive
causes increased deposition of extracellular matrix in the interstitium

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5
Q

What does the extracellular matrix consist of?

A

3D fibre mesh filled with macromolecules of collagen and elastin

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6
Q

What features does the extracellular matrix provide the lung with?

A

elasticity

low resistance for gas exchange

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7
Q

What are the three classifications of interstitial lung diseases?

A

environmental exposure
systemic inflammatory diseases
idiopathic

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8
Q

What are two examples of intersitial lung diseases caused by environmental exposure?

A

occupational lung disease

hypersensitivity pneumonitis

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9
Q

What are the two common causes of hypersensitivity pneumonitis?

A

mold

bird proteins

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10
Q

What are the three common causes of occupational lung disease?

A

silicosis
coal miners lung
asbestosis

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11
Q

What is silicosis caused by?

A

exposure to silica

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12
Q

What is coal miners lung caused by?

A

coal dust exposure

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13
Q

What is an example of an idiopathic intersitial lung disease?

A

Idiopathic pulmonary fibrosis

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14
Q

What are two examples systemic inflammatory ILDs?

A

connective tissue disease

sarcoidosis

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15
Q

What are the six main causes of intersitial lung disease?

A 
drugs
idiopathic
sarcoidosis
connective tissue disease
hypersensitivity pneumonitis
occupational lung diseases
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16
Q

What are the symptoms of intersitial lung disease?

A

slowly progressive exertional dyspnoea
non-productive cough
dry, inspiratory bibasal “Velcro” crackles
sometimes have clubbing of fingers
abnormal pulmonary function test results with restriction and impaired gas exchange

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17
Q

What are the four different types of patterns that can be seen on a lung HRCT?

A

basal distribution
subpleural
traction bronchiectasis
honeycombing

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18
Q

How can IPF be managed?

A

anti-fibrotic drugs
lung transplant
supportive care

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19
Q

How much do anti-fibrotic drugs cost?

A

£24,000 per person/yr

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20
Q

What are two examples of anti-fibrotic drugs?

A

pirfenidone

nintedanib

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21
Q

Why can anti-fibrotic drugs only be prescribed by specialist centres?

A

because they are very expensive

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22
Q

What is the function of anti-fibrotic drugs?

A

to slow rate of disease progression of the interstitial lung disease

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23
Q

What is the only criteria for being prescribed anti-fibrotic drugs?

A

that the patient has a diagnosis of idiopathic disease

that the patient has an FVC of between 50 - 80 % of their normal predicted FVC

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24
Q

Why are anti-fibrotic drugs often poorly tolerated?

A

significant weight loss
significant GI upset (with nausea and diarrhoea)
photosensitivity (have to wear sunblock at all times)

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25
Q

How is suitability assessed for a lung transplant?

A

usually under the age of 65
with no significant comorbidities
RARE to be suitable - ILB is a disease of the elderly

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26
Q

What are the common causes for deterioration in IPF patients?

A

infection
pulmonary embolism
pneumothorax
acute exacerbation

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27
Q

What is the only deterioration when IV steriods can be used for treatment in IPF patients?

A

when the patient develops acute exacerbation

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28
Q

What are some co-morbidities of IPF patients?

A

gastroesophageal reflux disease
COPD (from history of smoking)
lung cancer

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29
Q

How are IPF patient’s symptoms palliated?

A

assessed for ambulatory oxygen or for long term oxygen

pulmonary rehab

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30
Q

What happens during long term oxygen delivery?

A

oxygen is provided for 15 to 16 hours per day

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31
Q

What si the effect of long term oxygen delivery for an IPF patient?

A

reduces chronic hypoxia

reduces their risk of cor pumonale

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32
Q

Which two things are improved through pulmonary rehab?

A

lung capacity

quality of life

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33
Q

Why are benzodiazepines and opiates prescribed for IPF patients?

A

to help manage anxiety and the sensation of breathlessness

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34
Q

What is the effect of hypersensitivity pneumonitis?

A

diffuse inflammation of the lung parenchyma

especially in the upper lobes

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35
Q

What is the cause of hypersensitivity pneumonitis triggered by?

A

an inhaled antigen

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36
Q

What could hypersensitivity pneumonitis be triggered by?

A

bird fancier’s lung
farmers lung
aspergillus
could be anything (e.g. playing bagpipes not cleaning them) - 50% of cases where the causing antigen is unknown

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37
Q

What causes bird fancier’s lung?

A

avian proteins present in the dry dust of the droppings and sometimes in the feathers of a variety of birds

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38
Q

What are the three classes of hypersensitivity pneumonitis symptoms?

A

acute
subacute
chronic

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39
Q

What are the acute symptoms of hypersensitivity pneumonitis?

A 
shortness of breath
cough
fever
malaise
crackles within 4-6 hours of heavy exposure
often misdiagnosed as infection
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40
Q

What are the sub-acute symptoms of hypersensitivity pneumonitis?

A

gradual onset of symptoms

weight loss - common

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41
Q

What are the chronic symptoms of hypersensitivity pneumonitis?

A

insidious onset
history of acute episodes may be absent
even after antigen removal, symptoms may continue
may lead to irreversible fibrosis

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42
Q

What condition is likely to be responsible for most cases of hypersensitivity pneumonitis?

A

many years of continuous or intermittent inhalation of the inciting agent

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43
Q

What type of complex is produced during the precipitin test?

A

IgG antibody-antigen complexes

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44
Q

How is hypersensitivity pneumonitis diagnosed?

A

using the precipitin test

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45
Q

How is hypersensitivity pneumonitis diagnosed?

A

using the precipitin test

46
Q

What are the three disadvs of the precipitin test when diagnosing HP?

A

there are an infinite number of possible antigens so it might be imposssible to find an antigen to use in the precipitin test / might all produce negative results
the precipitin test may be positive in asymptomatic individuals
negative results does not exclude hypersensitivity pneumonitis

47
Q

How is HP managed?

A

once identified, the patient needs to avoid the inciting antigen
in early disease, is usually steroid-responsive (IV)

48
Q

What are three common drugs that could trigger ILD?

A

Nitrofurantoin
Amiodarone
Methotrexate

49
Q

What are four connective tissue diseases that are related to ILD?

A

Rheumatoid Arthritis
Scleroderma
Sjogrens
Polymyositis

50
Q

What group of people are connective tissue disease-related ILD common in?

A

Younger patients

Female preponderance

51
Q

What condition histories need to be taken from patients who might have connective tissue disease-related ILD?

A

dryness of the eyes or mouth (sicca)
Raynaud’s
joint pain/swelling
rashes

52
Q

How is connective tissue disease-related ILD managed?

A

managed in liasion with staff in rheumatology

the underlying disease needs to be treated

53
Q

Why are bloods done in new IDL patients?

A

to look for indications that it might be connective tissue disease-related ILD

54
Q

What two substances are found in the blood tests that suggest that the patient has connective tissue disease-related ILD?

A

antinuclear antibodies
rheumatoid factor
angiotensin-converting enzyme (sometimes)

55
Q

How is the underlying disease treated in connective tissue disease-related ILD? (bis)

A

biologics
immunosuppression
steroids

56
Q

What is the FEV1/FVC ratio expected to be in patients with ILD?

A

normal to high

57
Q

Which condition might confuse the lung function test results of ILD patients?

A

coexistent COPD

58
Q

What will be seen on the chest xray of an ILD patient?

A

reticular shadowing

59
Q

Why are HRCTs the cornerstone of diagnosing ILD patients?

A

bc the pattern of disease abnormality seen on the scan can inform on the likely aetiology

60
Q

What percentage of HRCTs are fully diagnostic?

A

60%

61
Q

What is the use of lung biopsies in ILD patients?

A

to try and differentiate from idiopathic pulmonary fibrosis which is irreversible
usually not done - elderly

62
Q

How is ILD treated pharmacologicaly?

A

immunosupression drugs

anti-fibrotics

63
Q

What immunosupression drugs can be used to treat ILD?

A

prednisolone
hydoxychloroquine
mycophenolate mofetil
methotrexate

64
Q

What type of disorder is sarcoidosis?

A

a multisystem granulomatous disorder

65
Q

How is sarcoidosis recognised?

A

the presence of non-necrotising granulomas found when a biopsy is performed on the affected tissues

66
Q

What is sarcoidosis caused by?

A

cause unknown

67
Q

What groups is sarcoidosis common in?

A

more common and severe in Afro-caribbeans
some familial clusters (a suggested genetic link)
middle aged people

68
Q

How does sarcoidosis differ for Afro-Carribeans?

A

three times more common
have more severe extra-thoracic symptoms
typically have more severe disease

69
Q

What age group is affected most by sarcoidosis? What percentage of total cases occurs in this age group?

A

30 - 60 year olds

75%

70
Q

What is the histological cause behind sarcoidosis?

A

an unknown antigen stimulates an immune response by CD4+ T cells and alveolar macrophages

71
Q

What is the result of the immune response triggered in sarcoidosis?

A

the CD4+ T cells and alveolar macrophages aggregate to form epithelioid cells and further antigens
stimulation then leads to further aggregation into multinucleate giant cells

72
Q

What happens to the multinucleate giant cells in sarcoidosis?

A

they organise into granulomas

73
Q

What is the difference between the granulomas present in sarcoidosis and those in TB or fungal infections?

A

in sarcoidosis, they are non necrotising / non caseating granulomas

74
Q

What is required to confidently diagnose sarcoidosis?

A

a biopsy demonstrating granulomas along with clinical picture

75
Q

How does sarcoidosis affect the skin?

A

erythema nodosum
lupus pernio
plaques
nodules

76
Q

What other systems of the body does sarcoidosis affect other than the lungs?

A

the skin
the eyes
the peripheral nerves

77
Q

How does sarcoidosis affect the eyes?

A

anterior uveitis

78
Q

Why might a sarcoidosis patient have had a lymph node biopsy?

A

patients with sarcoidosis may have significant systemic upset and can easily be confused with cancer or typically lymphoma
it would have been performed for a suspected malignancy
it’s not until the lymph node is removed and examined that they find these non necrotising granulomas

79
Q

How can sarcoidosis be recognised from a blood test?

A

a positive result for hypoglycaemia

80
Q

Why might a sarcoidosis patient have hypoglycaemia?

A

activated cells within sarcoid granulomas secrete the
enzyme which converts inactive vitamin D to its activated form
activated vitamin D is important for the absorption of calcium within the body
AAR patients with sore quote have increased activated vitamin D levels and hence hypoglycaemia

81
Q

How does sarcoidosis affect the peripheral nerves?

A

Bell’s Palsy

mononeuropathies

82
Q

What three conditions are included in the triad of lofgren syndrome?

A

Erythema Nodosum
Bilateral Hilar Lymphadenopathy
Arthralgia

83
Q

What are symptoms of lofgren syndrome beyond the triad?

A

systemic upset

fever

84
Q

Why is a diagnosis of Lofgren Syndrome not bad news?

A

it has an excellent prognosis
it’s usually self limiting
usualy has no need for any treatment

85
Q

When might pulmonary sarcoidosis be entirely asymptomatic?

A

if the sarcoidosis is isolated to the lymph nodes in the chest

86
Q

What are the lung-specific symptoms of pulmonary sarcoidosis?

A

cough
breathlessness with exertion
chest tightness

87
Q

What are the systemic symptoms of pulmonary sarcoidosis?

A 
fatigue (can be extremely difficult to treat)
sweats
weight loss 
fevers
can lead to lymphoma
88
Q

What happens to patients with pulmonary sarcoidosis?

A

majority - remission (with or without treatment)

minority - chronic inflammation continues and may eventually lead to fibrosis

89
Q

What combination is used to diagnose sarcoidosis?

A

clinical picture
exclusion of alternative diagnoses ( esp. lymphoma and TB)
ideally biopsy of affected tissue

90
Q

What do the baseline tests look at when diagnosing sarcoidosis?

A 
renal function
liver function
calcium
serum ACE
CXR
ECG
91
Q

What is serum ACE secreted by in sarcoidosis patients?

A

activated alveolar macrophages in granulomas

92
Q

Why is serum ACE not a reliable diagnostic identifier for sarcoidosis?

A

low sensitivity - only about 60% of patients will have serum ACE
poor specificity - serum ACE may be raised in patients who do not have sarcoidosis
peripheral blood ACE levels vary
there is no correlation with the chest xray stage of disease

93
Q

Why are there variations in peripheral blood ACE levels?

A

bc there are polymorphisms in the ACE gene

94
Q

When can it be beneficial to use serum ACE?

A

in some patients, monitoring the SACE can be a useful way of assessing disease activity

95
Q

How can it be beneficial to use serum ACE?

A

in some patients, there can be clear elevations of the serum ACE when their disease is active
then the levels normalise when in remission

96
Q

In what situations might systemic treatment be administered for sarcoidosis?

A

if the patient has cardiac sarcoidosis
if the patient has neurological sarcoidosis
if the patient has an ocular disease that is not responding to topical treatment

97
Q

What does “ocular disease not responding to topical treatment” mean?

A

if the patient has eye disease that is not responding to eyedrops

98
Q

What is the treatment plan for pulmonary sarcoidosis?

A

steroids improves the symptoms in the short-term
often less severe than extra-thoracic disease with spontaneous remission common so a lot of the time, no treatment needed
the long-term effect on natural history of disease not known

99
Q

How long are corticosteroids used to treat sarcoidosis patients?

A

6 months - 2 years

100
Q

Why are corticosteroids used to treat sarcoidosis patients?

A

inhaled corticosteroids may provide symptomatic benefit

AAR they might not need systemic treatment

101
Q

When might additional immunosuppressants be used to treat sarcoidosis patients?

A

when we’re unable to reduce the steroid dose bc the patient keeps getting flares of disease
very severe progressive disease from the outset

102
Q

What does a restrictive pattern from a spirometry indicate compared to that of an obstructive pattern?

A

restrictive - indicates that there is a problem

obstructive - indicates that there is a problem, specifically in the airways

103
Q

How is obstructive spirometry useful diagnostically?

A

for reversible airflow obstruction (asthma)

to indicate that there is fixed airflow obstruction in a smoker (COPD)

104
Q

How is restricted spirometry useful for?

A

monitoring the FEC

acts as a part of the treatment criteria for its empathic pulmonary fibrosis

105
Q

Which two conditions can obstructive spirometry be useful for monitoring change in?

A

acutely - Guillain-Barre

chronically - COPD?

106
Q

How is restricted spirometry useful for monitoring the FEC?

A

indicates the risk of impending ventilatory failure

indicates the severity of the ILD

107
Q

Why do patients get restrictive spirometry?

A

when lung expansion is restricted

when there is loss of lung volume

108
Q

What represents the total lung capacity?

A

FVC

109
Q

Why is getting the FVC value easy?

A

can be done in clinic

is useful for disease monitoring

110
Q

What can cause restrictive pattern spirometry?

A

Extensive burns, scleroderma (skin layer)
Raised BMI (subcutaneous tissue layer)
Kyphoscolios (thoracic cage layer)
e.g. MND, Guillain-Barre (neuromuscular system layer)
Pleural thickening (pleural layer)
Pneumonectomy, pleural effusion (caused by the loss of lung volume)
ILD (caused by lung parenchyma)

M103 T4 (19 decks)

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