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respiratory system year 1 > pathology of restrictive lung disease > Flashcards

pathology of restrictive lung disease Flashcards

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1
Q

what are restrictive lung diseases also known as

A

diffuse or interstitial lung disease

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2
Q

what area of the lung is most commonly involved in restricitve lung disease

A

the interstitium

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3
Q

what is the interstitium of the lung

A

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

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4
Q

describe the interstitium in healthy lungs

A

the normal alveolar wall, most of the alveolar epithelial and interstitial capillary endothelial cell beasement membranes are in direct contact

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5
Q

describe the changes to the alveolar wall in interstitial lung disease

A

alveolar wall thickened by interstitial infiltrate

inflammatory change –> fibrosis (production of tissue between the layers of the basement membrance, interferes with gas exchange)

introduction of collagen and fibrous tissue means the lungs become stiff and cannot stretch as easily

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6
Q

what is meant by reduced compliance

A

lungs are stiff and don’t expand as easliy

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7
Q

features of FEV1, FVC, gas transfer and V/Q ration in restrictive lung disease

A

low FEV1, low FVC
normal FEV1/FVC ratio
reduced gas transfer (diffusion abnormality)
V/Q imbalance when small airways are affected by pathology (not all conditions)

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8
Q

presentation of diffuse lung disease

A

discovery of abnormal CXR/CT

DYSPNOEA - on evertion or at rest

type 1 resp failure

heart failure - as a result of hypoxaemia and pulmonary vasoconstriction

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9
Q

what is the most common presentation of diffuse lung disease

A

dyspnoea

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10
Q

what is the difference between emphysema and interstitial lung disease on a CXR

A

emphysema - hyperinflated lungs

interstitial lung disease - reduced lung volumes, increased lung markings

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11
Q

pattern of development for interstitial lung disease

A 
parenchymal (interstitial injury) 
acute response --> chronic response
chronic response leads to one of 3 options: 
usual interstitial pnuemonitis
granulomatous response
other patterns

end result for all is fibrosis or end-stage honeycomb lung

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12
Q

what type of sensitivity is a granulomatous response

A

mix of type III and IV sensitivity

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13
Q

inflammatory conditions

A

inflammation is mostly chronic in the interstitium of the lung

can be acute which converts into chronic

there is relatively limited way in which the lung can respond to injury

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14
Q

what is diffuse alveolar damage

A

an altered form of acute inflammation

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15
Q

do all conditions lead to pulmonary fibrosis

A

NO

not all conditions result in scarring and fibrosis

pulmonary fibrosis is irreversible and may be fatal

some conditions are very rarely at risk of progressing this way

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16
Q

what is the mortality rate for DADS

A

> 50%

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17
Q

what does DADS stand for

A

diffuse alveolar damage syndrome

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18
Q

what changes occur in DADS

A

changes are related to alveolar epithelium and capillary endothelium

results in leaky vessels

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19
Q

what is DADS associated with

A 
major trauma e.g. cardiac arrest
chemical injury/toxic inhalation
circulatory shock 
drugs 
infection - DADS can complicate the effects of an infection
autoimmune disease
radiation

can also be idiopathic

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20
Q

what are the 2 stages of DADS

A

exudative stage - oedema (0-2 days following injury) due to leaky capillaries, plasma proteins precipitate in the lungs and form a layer (1-14 days)

proliferative stage (7 days) - interstitial inflammation and fibrosis

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21
Q

histological features of DADS

A

protein rich oedema
fibrin
hyaline membranes
denuded basement membranes

(lung is trying to repair itself after the damage but isn’t good at it)

epithelial proliferation
fibroblast proliferation
scarring - interstitium and airspaces

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22
Q

sarcoidosis

A

a multisystem granulomatous disorder of unknown aeitology

most likely affects lymph nodes and lungs

most likley one to encounter in clinical practice

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23
Q

histopathology of sarcoidosis

A

epithelioid and giant cell granulomas - accumulation of macrophages
necrosis/caseation (cheese like appearance) is very unusual
little lymphoid infiltrate
variable associated fibrosis

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24
Q

incidence of sarcoidosis

A 
commonly affects young adults 
f>m
3-4/100 000 in UK
20/100 000 in african-americans in USA
low in equatorial regions 
it is a disease of temperate climates
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25
organ involvement in sarcoidosis (% cases)
``` lymph nodes - almost 100 lung - >90 spleen - 75 liver - 70 skin, eyes, skeletal muscle - 50 bone marrow - 20 salivary glands - up to 50 ```
26
sarcoidosis presentation
1. young adult, acute arthralgia, erythema nodosum, bilateral hilar lymphadenopathy 2. incidental abnormal CXR/CT, asymptomatic 3. SOB, cough, abnormal CXR most (esp 1) resolve after 2yrs 2 and 3 may resolve, persist or progress
27
what is arthralgia
joint pain
28
what is erythema nodosum
nodular eruptions over the skin
29
what is the treatment for sarcoidosis
corticosteroids
30
sarcoidosis diagnosis
clinical findings imaging findings serum Ca++ and ACE biopsy (only needed in relatively few patients)
31
what is hypersensitivity pneumonitis
granulomatous response caused by inhalation of organic antigens
32
what antigens cause hypersensitivity pneumonitis
``` thermophilic actinomycetes (fungus): micropolyspora faeni, thermoactinomyces vulgaris bird/animal proteins - faeces, bloom fungi: aspergillus spp chemicals others e.g. drugs ```
33
what is hypersensitivity pneumonitis also known as
extrinsic allergic alveolitis
34
acute presentation of hypersensitivity pneumonitis
fever, dry cough, myalgia chills 4-9hrs after antigen exposure crackles, tachyopnoea, wheeze precipitating antibody high dose of the antigen can lead to infection-like presentation
35
chronic presentation of hypersensitivity pneumonitis
insidious malaise, SOB, cough low grade illness crackles and some wheeze can lead to resp failure, low gas transfer
36
histopathology of hypersensitivity pneumonitis
immune complex mediated combined type III and IV hypersensitivity reaction soft centriacinar epithelioid granulomata interstitial pneumonitis foamy histiocytes bronchiolitis obliterans
37
why is hypersensitivity pneumonitis an upper zone disease
the parts of the lung most affected are the areas where the inhaled material first lands laminar flow --> diffusion point, inhaled material is no longer carried and is deposited centriacinar regions of the lung are where most pathology occurs
38
which condition is most likely to progress to severe pulmonary firbosis
usual interstitial pneumonitis can be fatal and lead to resp failure poor prognosis
39
where may usual interstitial pneumonitis (UIP) be seen
``` connective tissue disease - esp scleroderma and rheumatoid disease drug reaction post-infection - viruses indusrtial exposure - asbestos others ```
40
what generally causes UIP
most are cryptogenic or idiopathic (generally cannot determine a possible cause) Idiopathic pulmonary fibrosis (IPF) or cryptogenic fibrosing alveolitis (CFA) don't say the patient has UIP, say they have IPF/CFA
41
histopathology of UIP
patchy interstitial chronic inflammation type II pneumocyte hyperplasia smooth muscular and vascular proliferation evidence of old and recent injury (temporal and spatial heterogeneity) proliferating fibroblastic foci
42
what is the importance of proliferating fibroblastic foci in UIP
they aren't unique to UIP manifestation of the repair and fibrosis process they are both a very important part of the pathological process
43
clinical example of idiopathic UIP
>50, m>f clinically show: dyspnoea, cough, basal crackles, cyanosis, clubbing progressive disease - most dead in 5yrs restrictive PFT and reduced gas transfer
44
what would a CXR oF UIP show
basal/posterior diffuse infiltrates cysts 'ground glass'
45
what is the prognosis for UIP
some fulminant some steroid responsive overall poor prognosis pulmonary fibrosis confers an increased risk of developing lung cancer
46
appearance of the lung in UIP
basal and posterior fibrosis and scarring with honeycombing creation of cystic spaces in the lung - byproduct of the attempt of the lung tissue to repair itself which has failed
47
normal pulmonary gas exchange
air flow in airways is laminar or turbulent, depends on pressure difference beyond terminal bronchiole - diffusion
48
how saturated is the blood leaving the capillary bed and why
98% saturated for FIO2 of 0.21 Hb affinity for oxygen
49
normal PaO2
10.5-13.5 kPa
50
normal PaCO2
4.8-6 kPa
51
type I respiratory failure
PaO2 <8 kPa | PaCO2 normal or low
52
type II respiratory failure
PaCO2 >6.5kPa | PaO2 usually low
53
what are the 4 abnormal states associated with hypoxaemia
alveolar hypoventilation shunt ventilation/perfusion imbalance diffusion impairment - generally doesn't lead to CO2 retention
54
alveolar hypoventilation
amount of air moved in and out of lungs hypoventilation increases PACO2 and thus increases PaCO2 PACO2 rise decreases PAO2 which causes PaO2 to fall fall in PaO2 due to hypoventilation is corrected by raising FIO2
55
V/Q mismatch
normal V/Q is 0.8 low V/Q is COMMONEST cause of hypoxaemia encountered clinically low V/Q in some alveoli arises due to local alveolar hypoventilation due to some focal disease hypoxaemia due to low V/Q responds well to small increases in FIO2
56
what does gas flow through a membrane depend on
thickness and SA of the membrane and gas pressure across it
57
how much faster does CO2 diffuse than O2 and why
20x | greater solubility
58
does diffusion impairment change CO2 levels
diseases impairing gas diffusion usually do no change CO2 levels it means it takes longer for blood and alveolar air to equilibrate, particularly for oxygen
59
how long does equilibration normally take
normally 0.25s in disease equilibration may take close to 0.75s
60
how long does capillary transit time take
0.75s at rest the time RBC spend in the alveolar capillary network
61
when may PaO2 falls occur in diffusion impairment disease
on exercise as capillary transit time falls exercise can precipitate hypoxaemia in interstitial lung disease
62
how can hypoxaemia by diffusion impairment be corrected
increasing FIO2 increases PAO2 therefore increasing rate of diffusion rarely clinically the sole cause of hypoxaemia
63
define shunt and normal values
blood passing from R to L side of heart w/o contacting ventilated alveoli normally 2-4% shunt
64
when can pathological shunt occur
AV malformations congenital heart disease pulmonary disease
65
do large shunts respond to increases in FIO2
they respond poorly | blood leaving the normal lung is already 98% saturated

respiratory system year 1 (25 decks)

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