clinical features and management of restrictive lung disease

Question 1

define restriction

Answer 1

forced vital capacity <80% of normal

Question 2

what is used as a marker of restriction

Answer 2

vital capacity

spirometry is key

Question 3

what are the causes of restriction

Answer 3

lungs 
pleura
nerve/muscle
bone 
other

Question 4

lung causes of restriction

Answer 4

interstitial lung disease:
idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis

Question 5

pleural causes of restriction

Answer 5

pleural effusion
pneumothorax
pleural thickening e.g. from TB (calcification)

Question 6

skeletal causes of restriction

Answer 6

kyphoscoliosis
ankylosing spondylitis
thoracoplasty
rib fractures - pain

Question 7

what is thoracoplasty

Answer 7

TB patients used to have ribs reduced/removed at the top to reduce lung volume in order to treat the TB before abx were developed

TB is aerobic so by reducing the lung volume it reduced the ability of the TB to grow

Question 8

muscular causes of restriction

Answer 8

amyotrophic lateral sclerosis - neurological cause of restrictive pattern to breathing

Question 9

sub-diaphragmatic causes of restriction

Answer 9

obesity

pregnancy

Question 10

is restrictive pattern of lung function always due to lung disease

Answer 10

NO

Question 11

Where is the interstitium

Answer 11

space between the epithelium of the alveolus and the endothelium of the capillary

1-2 cells thick over which gas exchange occurs

Question 12

what are interstitial lung diseases

Answer 12

> 200 disease causing thickening of the interstitium and can result in pulmonary fibrosis

Question 13

top 3 ILDs

Answer 13

sarcoidosis
idiopathic pulmonary fibrosis
hypersensitivity pneumonitis

Question 14

what is sarcoidosis

Answer 14

multisystem granulomatous disease of unknown cause

histological hallmark is non-caseating granuloma

just because there is a granuloma doesnt mean there is sarcoidosis

Question 15

what is erythema nodosum

Answer 15

bruising rash
well known association with sarcoidosis
lumps on the shins tend to be sore

Question 16

where are granulomas found in sarcoidosis

Answer 16

skin lesions

often locate to areas of trauma e.g. scars

Question 17

who gets sarcoidosis

Answer 17

adults <40
f>m
world-wide

Question 18

how is sarcoidosis investigated

Answer 18

Hx and exam

CXR

Question 19

stage I sarcoidosis disease on CXR

Answer 19

nodes only

lymphadenopathy and enlarged blood vessels

Question 20

stage II sarcoidosis disease on CXR

Answer 20

snowstorm shadowing

nodes and lung parenchymal involvement

Question 21

stage III sarcoidosis disease

Answer 21

fibrosis

Question 22

stage IV sarcoidosis disease

Answer 22

wrecked lung

irreversible w/o transplant

Question 23

how is sarcoidosis investigated

Answer 23

pulmonary function tests - can’t be done in isolation
bloods - lower lymphocyte conc in peripheral blood, concentrated in lungs
urinalysis - check for protein or blood, differentiate from vasculitis
ECG - cardiac sarcoidosis can be fatal
TB skin test - granulomas can also be TB
eye exam - check for posterior uveitis, anterior uveitis is bright red

Question 24

why are smokers less likely to get sarcoid

Answer 24

suppression of lymphocyte response

Question 25

further assessments for sarcoidosis

Answer 25

bronchoscopy including transbronchial biopsies and endobronchial ultrasound
granulomas can be seen
high res CT - snowstorm, tend to grow along lymphatics

Question 26

surgical biopsies

Answer 26

mediastinoscopy
video assissted thoracoscopic lung biopsy (VATS)

fairly rare, EBUS is more commonly used

may need to take out a node/piece of lung

Question 27

how does CXR affect prognosis of sarcoidosis (remission rate)

Answer 27

stage I 55-90%, enlarged glands - 90% chance of spontaneous resolution
stage II 40-70%
stage III 10-20%
stage IV 0%

Question 28

how is sarcoidosis treated

Answer 28

mild disease, no vital organ involvement, normal lung function, few symptoms - no treatment

erythema nodosum/arthralgia - NSAIDs

skin lesions/anterior uveitis/cough - topical steroids

cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia - systemic steroids

Question 29

what is the prognosis for sarcoidosis

Answer 29

<1% of caucasians die
10-205 sustain permanent pulmonary or extra-pulmonary complications

respiratory: progressive resp failure, bronchiectasis, aspergilloma, haemoptysis, pnuemothorax

Question 30

what is the main histological feature of idiopathic pulmonary fibrosis

Answer 30

fibroblastic focus

these areas produce collagen

Question 31

typical presentation of IPF

Answer 31

chronic SOB and cough
typically 60-70y/o
m>f
failed treatment for ‘LVF’ or infection

clubbed (40%) and crackles (can be fluid, infection or fibrosis)

Question 32

appearance of IPF of CXR

Answer 32

fluffy white shadowing at the lung bases

no sharp heart border, fluffy and merges with the lung

Question 33

appearance of IPF on CT

Answer 33

honeycombing round the edge of the lungs

tends to start at the edge and move inwards gradually

Question 34

median survival for IPF

Answer 34

3 yrs

Question 35

treatment for IPF

Answer 35

refer to ILD clinic
oral anti-fibrotic (OAF) - pirfenidone, nintedanib
palliative care

drugs slow down disease progression, don’t improve QOL (can be quite toxic)

surgical option = transplant (best option for <65 y/o)

Question 36

hypersensitivity pnuemonitis causes

Answer 36

birds
farming
malt whiskey

hx is important - make sure not to miss any potential exposures