L22- Restrictive Lung Disease Flashcards

1
Q

What 2 defects in restrictivr lead yo hypoxia in the end

A

Restriction of lung compliance ie elasticity and chest wall movement

Or

Impaired gas rxchnage tossue eg oedema filling or fiborsis to thicken it

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2
Q

Altered lung parenchyma is 1 cause of restticitvie disease. What is the parenchyma

A

Functional lung tissue eg for diffsuion, perfusion and ventilatiom

Eg alveoli, ducts and resp bronchioles

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3
Q

Give examples of sltered parenchyma

A

Fibrotic scarring increasing thickeneds

Honeycomb destroyed tissues reducing amount of cells for excusnhe

Fibrotic deposits clotting the blood capillaries

Oedema causing collapse and also reduced diffusion of gases

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4
Q

Disease of what fluid filled cavity can cuase restricififr disease and givr rxamples

A

Pleura

Eg pneumothorax when it fills with air

Haemothorax filling with blood
Empysema pus filled

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5
Q

How does pleura in heslthy krep lungs inflated allowing incressed capacity

A

Holds a nefstivr pressure below atmospheric

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6
Q

If it gets dilled what happens

A

Causes restrircuon in lung,collapsing, dysponea, cyanosis when hypxoia occurs

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7
Q

Which article dusccuses the pleura

A

Choi 2014

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8
Q

What did choi 2014 aay happens in pneumothroax

A

Air from slveoli floods into the pleura causinf incrrased pressure and tehrefore less inflated lungs

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9
Q

By what % does fvc decrease in pneumothorax meaning it’s restrictive

A

33%

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10
Q

Whats the difference between fluid filled pleura and air filled on xray

A

Fluid causes increased consolidation

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11
Q

Give 2 examples of chest wall abnormalities causing restrictive disease

A

Rib fracture, reduces lung ability to expand

Scoliosis too

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12
Q

What does nueormuscular disease causing restriction mean

A

Either the muscles are weajnfor resp or the nerves whcih control them cussing restriction

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13
Q

What is an exampke of this and give the article

A

Duchenne muscular dystrophy

Stehling et al 2015

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14
Q

Which muscles are weakened in this (stehling£

A

Expiratort

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15
Q

What does this lead to redcued

A

Reduced pef and cough peak flow. This means refuced fvc, fev1, increased infrctions due to lack of coughing and impaired resp failure

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16
Q

What 3 things lead to restricted gas exchnahe in these fiseases

A

Ability to expand lost eg neuormusc disease

Gas exchnage surfece loss ie crll loss

Reduced gas exchnage efficiency eg oedema ot fibrotic depoditd

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17
Q

What can cause abnormal v/q in restrictive diseases

A

If there is damage to both epithelium and vasculature eg fibrosis reduces capillaries

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18
Q

What is stiff lung due to causing lsck of compliance

A

Loss of parenchyma= damage elasticity, fiborsis increase, increased interstitail fluid

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19
Q

Where is interstitium gas exchange surfsce

A

Between capillaru endothelium and the alveolar basemment membrane

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20
Q

What is reduced in rds in infants

A

Surfsctant causing alveolar collapse

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21
Q

Which zone do type 1 have squahsed organelles

A

Perinuclear zone

22
Q

Where do the organelles products diffuse into

A

The cytoplasmic flap of type 1

23
Q

If macrophages dont exit vis mucocilatort. How

A

Exit cis the blood/lymph shen crossing interstitium

24
Q

How many people in uk affected by interstitial lung diseases

A

1 in 2000 (v rare)

25
What % caused by environemtn eg dusts
35
26
What are the causes of chronic ild like idiopathic pf
Idiopsthic ie unknown trigger Hypersensitivity to dust Immune response to infections Autoimmune eg ra
27
Who usually more likely to get ipf
Men and okder acove 70
28
What are rhe desth toll each year in uk by ipf
2000
29
Cystic airspaces in endstage honeycombinf (3-10mm) is filled with what
Mucus and inflam cells where notmal tossue used to be
30
What is spatial and temporal heterogeneity of ipf
Occurs over a long time and some places cna be heslthy vs diseased = spatial. Temporal means at diff stages eg some areas are actively depositing whereas others are already scar tissue
31
Lung tissue damage cia injury eg dust is the trigger. This causes inflammation and in what sites
Bronchioles = bronchiolitis Alveoli= alveolitis Capillaries = vasculitis
32
What soes inflammation allow
Finrosis to occur or oedema if ards
33
What sort of profibrotic cytokines are released by damaged epi crlls during trigger in ild
Tgfb, pdgf, tnfa
34
What does tgfb reduce which increases levels of collagen
Mmps
35
Where does te fibrotic deosits go
In interstitium disrupting it
36
What do myofibroblasts do when fibroblasts diff into them
Excess secretion of fibrous proteins like collagen = progressive lining stiffening/density
37
Which 2 released were found to increasde angiogenesis even though ipf refuces capillaries
Fgf2 and vegf
38
Which article discusses pathogensis of ipf
King jr et al 2011
39
Which comorbidities adsoicated with fibrosis
Obedity, siabetes, bronchiolitis, emphysema
40
What aort of effects do microinjuires like smoke or dust have on the epi cells provokinf the ipf response
Cell dearh
41
How does myofibroblasts stimulate more of the ipf response
They induce more apoptosis of aec
42
What usually in nfomal wound healing happens to myofibroblasts
Get apoptosed. Doesnt happen in ipf
43
What stops mf apoptosing so more fibrosis occurs
Tgfb. Forms resistant myofibroblasts
44
Whixh wnzymes are increased wgich allow viild up of thingns like collagen
Timps which block mmps
45
Why is there loss of thpe 1 cells in ipf permanently
The transition from type 2 to 1 is inhibited by bm damage and ecm
46
What does tfb1 promote in terms of cells in ipf
Myofibroblasts transition and emt too
47
What mmps acticate tgf B makinf thrm profibrotic
Mmp 2 and 9
48
Which cells produce these
Neutrophils, macrophages and fibroblasts themselves. (All recruited in inflammation /by damaged cells)
49
Which Moniclonal ab treatment showrd to worsen ipf
Simtuzimab
50
And which drug
Pirfenidine
51
What tewo category drugs given for ipf
Anti fibrotic and anti inflam
52
Why are antacids given
Because acid can cause epithelial damage causing ipf