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Mucosal Surfaces > L22- Restrictive Lung Disease > Flashcards

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
Q

What % caused by environemtn eg dusts

A

35

26
Q

What are the causes of chronic ild like idiopathic pf

A

Idiopsthic ie unknown trigger
Hypersensitivity to dust
Immune response to infections
Autoimmune eg ra

27
Q

Who usually more likely to get ipf

A

Men and okder acove 70

28
Q

What are rhe desth toll each year in uk by ipf

A

2000

29
Q

Cystic airspaces in endstage honeycombinf (3-10mm) is filled with what

A

Mucus and inflam cells where notmal tossue used to be

30
Q

What is spatial and temporal heterogeneity of ipf

A

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
Q

Lung tissue damage cia injury eg dust is the trigger. This causes inflammation and in what sites

A

Bronchioles = bronchiolitis

Alveoli= alveolitis

Capillaries = vasculitis

32
Q

What soes inflammation allow

A

Finrosis to occur or oedema if ards

33
Q

What sort of profibrotic cytokines are released by damaged epi crlls during trigger in ild

A

Tgfb, pdgf, tnfa

34
Q

What does tgfb reduce which increases levels of collagen

A

Mmps

35
Q

Where does te fibrotic deosits go

A

In interstitium disrupting it

36
Q

What do myofibroblasts do when fibroblasts diff into them

A

Excess secretion of fibrous proteins like collagen = progressive lining stiffening/density

37
Q

Which 2 released were found to increasde angiogenesis even though ipf refuces capillaries

A

Fgf2 and vegf

38
Q

Which article discusses pathogensis of ipf

A

King jr et al 2011

39
Q

Which comorbidities adsoicated with fibrosis

A

Obedity, siabetes, bronchiolitis, emphysema

40
Q

What aort of effects do microinjuires like smoke or dust have on the epi cells provokinf the ipf response

A

Cell dearh

41
Q

How does myofibroblasts stimulate more of the ipf response

A

They induce more apoptosis of aec

42
Q

What usually in nfomal wound healing happens to myofibroblasts

A

Get apoptosed. Doesnt happen in ipf

43
Q

What stops mf apoptosing so more fibrosis occurs

A

Tgfb. Forms resistant myofibroblasts

44
Q

Whixh wnzymes are increased wgich allow viild up of thingns like collagen

A

Timps which block mmps

45
Q

Why is there loss of thpe 1 cells in ipf permanently

A

The transition from type 2 to 1 is inhibited by bm damage and ecm

46
Q

What does tfb1 promote in terms of cells in ipf

A

Myofibroblasts transition and emt too

47
Q

What mmps acticate tgf B makinf thrm profibrotic

A

Mmp 2 and 9

48
Q

Which cells produce these

A

Neutrophils, macrophages and fibroblasts themselves. (All recruited in inflammation /by damaged cells)

49
Q

Which Moniclonal ab treatment showrd to worsen ipf

A

Simtuzimab

50
Q

And which drug

A

Pirfenidine

51
Q

What tewo category drugs given for ipf

A

Anti fibrotic and anti inflam

52
Q

Why are antacids given

A

Because acid can cause epithelial damage causing ipf

Mucosal Surfaces (33 decks)

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