restrictive lung diseases Flashcards

(46 cards)

1
Q

what is meant by the interstitium of lung?

A

connective tissue space around alveoli

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2
Q

what are some clinical symptoms caused by interstitial lung diseases?

A

reduced lung compliance
low FEV1 and FVC but usually normal ratio
reduced gas transfer
ventilation perfusion imbalance

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3
Q

how would restrictive interstitial lung disease discovered?

A

usually by imaging but can also present in patients as dyspnoea upon exertion and at rest as disease progresses

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4
Q

what are the 3 main types of interstitial lung disease ?

A

sarcoidosis
diffused alveolar damage (DAD)
hypersensitivity pneumonitis

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5
Q

what are the histological features of DAD?

A

fibrin
protein rich oedema
hyaline membranes

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6
Q

whats DAD associated with?

A

chemical injury
circulatory shock
drugs
infections/viruses

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7
Q

what are the histological features of sarcoidosis?

A

giant granuloma formation

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8
Q

what causes hypersensitivity pneumonitis caused?

A

hypersensitive reaction to organic molecules or antigens

eg. animals, birds & micro-organisms

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9
Q

whats really important about hypersensitivity pneumonitis?

A

taking a history to find out if the patient has been exposed to organic molecules or antigens

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10
Q

where does hypersensitivity pneumonitis begin ?

A

centriacinar region

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11
Q

what could hypersensitivity pneumonitis present as?

A

acute pneumonia but it is more chronic and it gradually deteriorates lung function

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12
Q

what do interstitial lung diseases end in?

A

end stage fibrosis

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13
Q

whats the normal PaO2 and PaCO2 range?

A

PaO2- 10.5-13.5kPA

PaCO2- 4.8-6kPA

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14
Q

what stats would define type 1 and 2 respiratory failure?

A

type 1- PaO2 < 8kPA (PaCO2 normal or low)

type 2- PaCO2 > 6.5kPA (PaO2 low)

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15
Q

what are some causes of hypoxaemia?

A

ventilation/perfusion imbalance
diffusion impairment
alveolar hypoventilation
shunt

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16
Q

what is the most common clinical cause for hypoxaemia?

A

ventilation/perfusion imbalance

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17
Q

what is meant by diffusion impairment?

A

does not change CO2 levels

takes longer for blood and alveolar air to equilibrate

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18
Q

whats the difference in the ventilation of abnormal alveoli between ventilation/perfusion imbalance and shunt?

A

ventilation/perfusion imbalance- some ventilation of abnormal alveoli
shunt- no ventilation of abnormal alveoli

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19
Q

how would you clinically define restriction?

A

forced vital capacity <80%

low spirometry volumes

20
Q

what body parts cause restrictive lung disease?

A

lungs
pleura
skeletal
muscle

21
Q

what are the lung causes for restrictive lung disease?

A

IPF
sarcoidosis
hypersensitivity pneumonitis

22
Q

what are the pleural causes for restrictive lung disease?

A

effusions
pneumothorax
pleural thickening

23
Q

what are the skeletal causes for restrictive lung disease?

A

kyphoscoliosis
rib fractures
ankylosing spondylitis (kampoura)

24
Q

what does a granuloma look like in histology?

A

rugby ball shaped collection of seeds

25
what tests would you do to diagnose sarcoidosis?
``` pulmonary function tests bloods urinalysis ECG eye exam ```
26
whats the treatment for sarcoidosis?
in increasing severity: -NSAIDS(non steroidal anti-inflammatories) - topical steroids - systemic steroids
27
whats a significant clinical symptom of IPF?
crackles and clubbing
28
w=how would IPF be treated?
usually palliative can be given oral anti-fibrotics and some patients may be eligible for transplant
29
what is obstructive sleep apnoea syndrome?
recurrent episodes of upper airway obstruction leading to apnoea during sleep, associated with heavy snoring, daytime sleepiness an poor concentration
30
how would OSAS be diagnosed?
clinical history and examination epworth questionnaire (score of >11 is abnormal) overnight sleep study
31
whats the treatment for OSAS?
- identify exacerbating factors eg. weight reduction, avoidance of alcohol, diagnose and treat endocrine disorders if present - continuous positive airway pressure (CPAP) - mandibular repositioning splint
32
what are the clinical features of narcolepsy?
cataplexy excessive daytime somnolence hallucinations sleep paralysis
33
how would you confirm a narcolepsy diagnosis?
polysonography multiple sleep latency test (MSLT) low orexin levels in CSF
34
what medications would be given to treat narcolepsy?
modafixil dexamphetamine venlafaxine (for cataplexy)
35
what are the clinical sats signs of chronic ventilatory failure?
elevated pCO2 pO2<8kPA normal blood pH elevated bicarbonate (HCO3)
36
whats the aetiology for chronic ventilatory failure ?
airway disease chest wall abnormalities respiratory muscle weakness central hypoventilation
37
what are the symptoms for chronic ventilatory failure?
``` breathlessness while standing up and lying flat ankle swelling morning headache recurrent chest infections disturbed sleep ```
38
what would be a key examination finding of chronic ventilatory failure?
paradoxical abdominal wall motion and ankle oedema
39
whats the treatment for chronic ventilatory failure?
non invasive ventilation | oxygen therapy
40
what tests would you carry out to support a chronic ventilatory failure diagnosis?
lung function tests ad assessment of hypoventilation | eg. lying/standing VC, early morning ABG, overnight oximetry
41
when are congenital abnormalities picked up?
they are present at birth and can be picked up at antenatal screenings (ultrasound,MRI) or in the newborn period
42
laryngomalacia
abnormal collapse of larynx presents with stridor, worse when feeding usually improves within the first year concern if it affects feeding, sleeping or growth
43
tracheomalacia
may be caused by external compression (vessels, tumour) associated with genetic conditions barking cough, recurrent croup, breathless at exertion and stridor/wheeze resolves naturally may require physio or antibiotics when unwell
44
trachea-oesophageal fistula
abnormal connection between trachea and oesophagus choking, colour change, unable to pass nasogastric tube surgical repair required
45
why does respiratory distress syndrome occur ad how is it treated?
occurs because of surfactant deficiency | antenatal steroids, surfactant replacement and appropriate ventilation & nutrition
46
what is meant by remodelling?
alteration or airway structure allowing external influence which leads to abnormalities