restrictive lung disease Flashcards

1
Q

what is restrictive lung disease ?

A

disease where there is a reduced ability of the lungs to expand and take in air.

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2
Q

what can restrictive lung disease be due to ?

A

reduced elasticity of the lung
impairment of the chest wall expanding

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3
Q

what are the two types of restrictive lung disease ?

A

intrinsic - problem is within lung
extrinsic - problem is outside the lung

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4
Q

what is the main type of intrinsic cause of restrictive lung disease ?

A

interstitial lung disease

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5
Q

why does extrinsic lung disease occur ?

A

restrictive spirometry
obesity
kyphosis
neuromuscular disease

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6
Q

what is interstitial lung disease ?

A

a group of lung disorders, characterised by inflammation and fibrosis, that make it hard for the lungs to get enough oxygen

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7
Q

how would a patients spirometry appear ?
include FVC, ratio etc.

A

present with a restrictive pattern on spirometry

FVC is reduced while FEV1 is proportionally normal

FEV1/FEV ratio is normal or may even be increased

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8
Q

what are the main symptoms of interstitial lung disease ?

A

breathlessness and a dry cough
usually progressive

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9
Q

what is the most common interstitial lung disease ?

A

Idiopathic pulmonary fibrosis

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10
Q

what is the definition of idiopathic pulmonary fibrosis ?

A

progressive pulmonary fibrosis with no clear cause.

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11
Q

where is idiopathic pulmonary fibrosis common ?

A

in smokers
usually affects those over 50

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12
Q

how does idiopathic pulmonary fibrosis present clinically and on examination ?

A

breathlessness and dry cough

on examination:
finger clubbing
bi-basal fine inspiratory crackles

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13
Q

what is first line diagnosis for IPF and what are the findings ?

A

CXR - lung infiltrates, fibrotic shadowing at periphery and bases of lungs

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14
Q

what is the second line diagnosis for IPF and what are the findings ?

A

high resolution CT thorax - classic ‘honeycomb’ appearance

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15
Q

what is the gold standard diagnostic test for IPF and what are the findings ?

A

lung biopsy

findings - destructive fibrosis with no/minimal inflammatory signs)

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16
Q

how does IPF develop ?

A

poor prognosis - life expectancy 2-5 years

17
Q

why are treatment options limited for fibrosis ?

A

it is irreversible

18
Q

what drugs is given for IPF to slow down fibrotic process ?

A

nintedanib
pirfenidone