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restrictive lung diseases Flashcards

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
Q

what tests would you do to diagnose sarcoidosis?

A 
pulmonary function tests
bloods
urinalysis
ECG
eye exam
26
Q

whats the treatment for sarcoidosis?

A

in increasing severity: -NSAIDS(non steroidal anti-inflammatories)

                                  - topical steroids
                                  - systemic steroids
27
Q

whats a significant clinical symptom of IPF?

A

crackles and clubbing

28
Q

w=how would IPF be treated?

A

usually palliative can be given oral anti-fibrotics and some patients may be eligible for transplant

29
Q

what is obstructive sleep apnoea syndrome?

A

recurrent episodes of upper airway obstruction leading to apnoea during sleep, associated with heavy snoring, daytime sleepiness an poor concentration

30
Q

how would OSAS be diagnosed?

A

clinical history and examination
epworth questionnaire (score of >11 is abnormal)
overnight sleep study

31
Q

whats the treatment for OSAS?

A
  • 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
Q

what are the clinical features of narcolepsy?

A

cataplexy
excessive daytime somnolence
hallucinations
sleep paralysis

33
Q

how would you confirm a narcolepsy diagnosis?

A

polysonography
multiple sleep latency test (MSLT)
low orexin levels in CSF

34
Q

what medications would be given to treat narcolepsy?

A

modafixil
dexamphetamine
venlafaxine (for cataplexy)

35
Q

what are the clinical sats signs of chronic ventilatory failure?

A

elevated pCO2
pO2<8kPA
normal blood pH
elevated bicarbonate (HCO3)

36
Q

whats the aetiology for chronic ventilatory failure ?

A

airway disease
chest wall abnormalities
respiratory muscle weakness
central hypoventilation

37
Q

what are the symptoms for chronic ventilatory failure?

A 
breathlessness while standing up and lying flat
ankle swelling
morning headache
recurrent chest infections
disturbed sleep
38
Q

what would be a key examination finding of chronic ventilatory failure?

A

paradoxical abdominal wall motion and ankle oedema

39
Q

whats the treatment for chronic ventilatory failure?

A

non invasive ventilation

oxygen therapy

40
Q

what tests would you carry out to support a chronic ventilatory failure diagnosis?

A

lung function tests ad assessment of hypoventilation

eg. lying/standing VC, early morning ABG, overnight oximetry

41
Q

when are congenital abnormalities picked up?

A

they are present at birth and can be picked up at antenatal screenings (ultrasound,MRI) or in the newborn period

42
Q

laryngomalacia

A

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
Q

tracheomalacia

A

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
Q

trachea-oesophageal fistula

A

abnormal connection between trachea and oesophagus
choking, colour change, unable to pass nasogastric tube
surgical repair required

45
Q

why does respiratory distress syndrome occur ad how is it treated?

A

occurs because of surfactant deficiency

antenatal steroids, surfactant replacement and appropriate ventilation & nutrition

46
Q

what is meant by remodelling?

A

alteration or airway structure allowing external influence which leads to abnormalities

Respiratory system (20 decks)

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