pathology of restrictive lung disease Flashcards

1
Q

what is the interstitium of the lung

A

the conective tissue soace around the airways and vessels and the soace between the basement membtanes of alveolar walls

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2
Q

what are the featires of restrictive diffuse interstitial lung disease

A

reduced lung compliance - stiff lungs
low FEV1 and low FVC but FEV1/FVC normal ratio
reduced gas transfer (Tco or Kco)
- diffusion abnormality
ventilation/perfusion imbalance
- when small airways affected by pathology

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3
Q

how does diffuse lung disease present

A

discovery of abnormal CXR or CT
dyspnoea
- shortness of breath on exertion
- shortness of breath at ready

resp failure - type 1
heart failure

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4
Q

what are chronic responses as a resukt to parenchymal (interstitial) lung injury

A

usual intersitial pneumonitus (UIP)
granulomatous responses
other patterns

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5
Q

what is an example of an acute response

A

diffuse alveolar damage (DAD)

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6
Q

what is DAD associated with

A
makor truama 
chemical injury/toxic inhalatjon 
circulatory shock 
drugs
infection including viruses 
auto(immune) disease 
radiation
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7
Q

what are histological features of DAD

A
protein rich oedema 
fibrin 
hyaline membranes
denudes basement membranes 
epithelial proliferation 
fribroblast proliferation 
scarring - interstitium and airspaces
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8
Q

what are examplea of acite granulomatous response

A

sarcoidosis

hypersensitivity pneumonitis

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9
Q

what is sarcoidosis

A

a multisystem granulomatous disorder of unknown aetiology

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10
Q

what is the histopathology of sarcoidosis

A
epitheliod and giant cell granulomatous 
necrosis/ caseation very unusual
little lymphoid infiltrate 
variable associated fibrosis 
type 4 hypersensitivity
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11
Q

what organs are involved in sarcoidosis

A
lymph nodes 
lung
spleen 
liver
skin, eyes, skeletal muscle
bone marrow 
salivary glands
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12
Q

how does sarcoidosis present

A
acute arthralgia 
erythema nodosum 
bikateral hikar lymphadenopathy 
incidental abnormal CXR or CT
SOC, cough and abnormal CXR
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13
Q

how do you disgnose sarcoidosis

A
clinical findings 
imaging 
serum calcium and ACE 
biopsy 
kveim test 
tuberculin test
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14
Q

what is hypersensitivity pneumonitis

A
reaction to organic molecules or antigens 
thermophilic actinomycetes - farmers lung
- micropolyspora faeni 
- thermoactinomyces vulgaris 
bird animal proteins - faces and bloom
fingu - asoergillus spp 
chemicals
others
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15
Q

what is the acute oresentation of hypersensitivity pneumonitis

A
fever 
drt cough
myalgia 
chills 4-9 hds aftr ag exposure 
crackles, tacgypnoea, wheeze 
precipitating antibody
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16
Q

what is the chronic presentation of hypersensitivity pneumonitis

A
insidious 
malaise 
SOB
cough 
low grade illness 
crackles and some wheeze
17
Q

what is the histopathology of hypersensitivity pneumonitis

A

immune complex mediated combined type 3 and 4 hypersensitive reactjon
soft centriacinar epitheliod granulomata
interstitial pneumonitis
foamy histoocytes
bronchiolitis obliterans
upper zone disease

18
Q

what is a thoe of UIP

A

isiopathic pulmonary fibrosis

19
Q

wbere is UIP found

A

may be seen in connective tissue disease
drug reaction
post infection
industrial exposure
most are idiopathic - hence idiopathic fibrosis

20
Q

what do chronsic reactions lead to in restrictive lung disease

A

honeycomb lung

21
Q

what is the histopathology of UIP

A
patchy interstitual chronic inflammation 
type 2 pneumocyte hyoerplasia 
smooth muscle amd vascukar proliferagion
evidence of old and recent injiry 
- temporal heterogeneity 
- spatial heyerogeneity 

proliferating fibroblastjc foci

22
Q

clinicak presentations of UIP

A
over 50s 
dyspnoea 
cough
basal crackles
cyanosis 
clubbing 
progressuve disease most dead in 5 yrs 
Cxr 
restruced PFT and reduced gas trabsger
poor prognosis