pathology of restrictive lung disease Flashcards

1
Q

what is The Interstitium of the Lung

A

The connective tissue space
around the airways and vessels
and the space between the basement membranes of the alveolar walls

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2
Q

should the alveolar epithelial and interstitial capillary endothelial cell basement membranes be in contact or apart?

A

contact

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3
Q

what does reduced lung compliance mean in the lungs?

A

stiff lungs

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4
Q

what does reduced gas transfer in the lungs indicate?

A

diffusion abnormality

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5
Q

what would ventilation/perfusion imbalance indicate?

A

when small airways affected by pathology

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6
Q

if a patient has restrictive lung disease, would the FEV1/FVC be lower or higher than normal?

A

higher

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7
Q

if a patient has restrictive lung disease, would the FVC be lower or higher than normal?

A

lower

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8
Q

whats the presentation of lung disease?

A

Discovery of Abnormal CXR

DYSPNOEA
Shortness of Breath on exertion
Shortness of Breath at rest

Respiratory Failure – Type 1
Heart Failure

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9
Q

what are the outcomes of parenchymal (interstitial) lung injury

A

acute response

chronic response

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10
Q

what are the outcomes of chronic response

A

usual interstitial pneumonitis UIP
granulomatous response
other patterns

ultimately ending up at fibrosis or end stage honeycomb

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11
Q

what is the acute response of interstitial lung injury?

A

diffuse alveolar damage

DAD

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12
Q

what is DAD associated with?

A
major trauma
chemical injury/toxic inhalation
circulatory shock
drugs
injection]auto(immune) disease
radiation
can be idiopathic
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13
Q

histological features of DADS

A
Protein rich oedema
Fibrin
Hyaline membranes
Denuded basement membranes
Epithelial proliferation
Fibroblast proliferation
Scarring - interstitium and airspaces
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14
Q

what are the two types of graanulomatous response?

A

sarcoidosis

hypersensitivity pneumonitis

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15
Q

what is sarcoidosis

A

A multisystem granulomatous disorder

of unknown aetiology

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16
Q

whats the histopathology of sarcoidosis?

A

epithelioid and giant cell granulomas
necrosis
little lymphoid infiltrate

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17
Q

what is the incidence of sarcoidosis in UK

A

3-4/100,000

18
Q

what is the most likely organ involved in sarcoidosis?

A

lymph nodes and lung

19
Q

whats the presentation of sarcoidosis

A

young adult
abnormal CXR- no sympt
sob, cough abnormal CXR

20
Q

how do you diagnose sarcoidosis?

A

Clinical findings
Imaging findings
Serum Ca++ and ACE
Biopsy

21
Q

what type of hypersensitivity gives you granulomatous responses?

A

type 4

22
Q

what are the antigens of hypersensitivty pneumonitis?

A
Thermophilic actinomycetes
Micropolyspora faeni
Thermoactinomyces vulgaris
Bird / Animal proteins - faeces, bloom
Fungi  - Aspergillus spp
Chemicals
Others
23
Q

what is acute presentation of hypersensitivity pneumonitis?

A

Fever, dry cough, myalgia,
Chills 4-9 hours after Ag exposure
Crackles, tachyopnoea, wheeze
Precipitating antibody

24
Q

what are the chronic presentation of hypersensitivity pneumonitis?

A

Insidious
Malaise, SOB, cough
Low grade illness
Crackles and some wheeze

25
Q

is hypersensitivity pneumonitis type 3 or 4 mediated?

A

both, 3 and 4

26
Q

what can cause usual interstitial pneumonitis?

A

idiopathic pulmonary fibrosis
connective tissue diseaases
drugs, asbestos, viruses

27
Q

what type of pneumocyte hyperplasia contributes to usual interstitial pneumonitis

A

2

28
Q

is UIP chronic or acute inflammation?

A

chronic

29
Q

what age would you normally be to get idiopathic pulmonary fibrosis?

A

elderly >50

30
Q

what gender gets pulmonary fibrosis more?

A

M>F

31
Q

what do people clinically show with pulmonary fibrosis

A

dyspnoea, cough, basall crackles, cyanosis, clubbing

32
Q

what 2 options can the bulk flow be?

A

laminar or turbulent

33
Q

does hypoventilation increase or decrease paCO2

A

increase

34
Q

what is the most common cause of hypoxaemia?

A

low V/Q (ventilation/perfusion)

35
Q

does CO2 or oxygen diffuse faster?

A

CO2 diffuses 20 times faster than o2

36
Q

why does co2 diffuse faster than o2?

A

greater solubility

37
Q

what does diffusion impairment mean?

A

takes longer for blood and alveolar air to equilibriate , particulary oxygen

38
Q

how long does equilibration normally takes?

A

0.25

39
Q

how long does capillary transit time normally take?

A

0.75

40
Q

how long does equilibration take in disease?

A

0.75

41
Q

how can hypoxaemia be corrected?

A

increasing FIO2

42
Q

what does increasing the FIO2 do to PAO2?

A

increases it