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Systems: Resp. WK > 25. Pathology of Restrictive Lung Disease > Flashcards

25. Pathology of Restrictive Lung Disease Flashcards

1
Q

What is the interstitium of the lung (pulmonary interstitum)?

A
  • The connective tissue (support tissue) space around airways and vessels and the space between basement membranes of the alveolar walls
  • includes alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and periymphatic tissues
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2
Q

In a normal alveolar wall, what two of its components are in direct contact?

A

most of alveolar epithelium (pneumocyte) and interstitial capillary endothelial cell basement membranes

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3
Q

What do all interstitial disease cause?

A

Cause thickening of the pulmonary interstitium which can be due to:

  • inflammation
  • scarring
  • extra fluid
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4
Q

Are all interstitial lung disease restrictive?

A

Yes

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5
Q

What do restrictive lung disease cause? (such as interstitial lung disease)

A
  1. Reduced lung compliance/expansion (stiff lungs therefore less volume of air)
  2. Low FEV1 and low FVC but FEV1/FVC has normal ratio
  3. Reduced gas transfer (diffusion abnormality)
  4. Ventilation/Perfusion imbalance (when small airways affected by pathology)
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6
Q

What is another name for interstitial lung disease?

A

Diffuse lung disease

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7
Q

How can interstitial/diffuse lung disease present itself? (4)

A
  1. Discovery of abnormal chest x ray
  2. dysponea
    (shortness of breath on exertion and shortness of breath at rest)
  3. Respiratory failure type 1
  4. heart failure
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8
Q

What does emphysema (obstructive) and interstitial lung disease (restrictive) do to the lungs?

A
  • emphysema expands them (hyperinflates chest)

- interstitial lung disease compresses them (lungs are stiff)

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9
Q

What are 2 types of parenchymal (interstitial) lung injury?

A
  1. acure response

2. chronic response

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10
Q

What does acute response in interstitial lung disease cause?

A

DAD: diffuse alveolar damage

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11
Q

What are possible causes of DAD (diffuse alveolar damage) which are due to acute response of interstitial lung disease? (7)

A
  1. major trauma
  2. chemical injury/toxic inhalation
  3. circulatory shock
  4. drugs
  5. infection
  6. auto(immune) disease
  7. radiation
    BUT it can be idiopathic (without known cause)
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12
Q

In DAD (diffuse alveolar damage), what 2 things arise in the first exudative stage?

A
  1. oedema

2. hyaline membranes (composed of proteins and dead cells line alveoli and prevent normal gas exchange)

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13
Q

In DAD (diffuse alveolar damage), what 2 things arise in the second proliferative stage?

A
  1. interstitial inflammation

2. interstitial fibrosis

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14
Q

What are 2 main stages in the evolution of DAD (diffuse alveolar damage)?

A
  1. exudative stage

2. proliferative stage

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15
Q

What are histological features of DADs? (diffuse alveolar damage: ACUTE RESPONSE) (7)

A
  1. protein rich oedema
  2. fibrin (when thrombin acts on fibrinogen it forms fibrin which clots blood)
  3. hyaline membranes
  4. denuded (stripped) basement membranes
  5. epithelial proliferation
  6. fibroblast proliferation
  7. scarring (interstitium and airspaces)
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16
Q

What is sarcoidosis?

A
  • A multisystem granulomatous disorder of unknown aetiology
  • CHRONIC response
  • granulomas (red, swollen tissues: granulomas develops on organs)
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17
Q

What is the histopathology of sarcoidosis? ( chronic response)

A
  • epitheliod and giant cell granulomas
  • necrosis/ caseation very unusual
  • little lymphoid infiltrate
  • variable associated fibrosis
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18
Q

Who is more likely to suffer from sarcoidosis? (chronic granulomatous response)

A
  • Commonly affects young adults
  • Affects females more than males
  • higher in prevalence in Afro-american in US and lower in equatorial regions
  • disease common in temperate/mild climates
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19
Q

What is lymph node involvement in sarcoidosis?

A

almost 100%

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20
Q

What is lung involvement in sarcoidosis?

A

> 90%

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21
Q

What is spleen involvement in sarcoidosis?

A

75%

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22
Q

What is liver involvement in sarcoidosis?

A

70%

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23
Q

What is skin, eyes and skeletal muscle involvement in sarcoidosis?

A

50%

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24
Q

What is bone marrow involvement in sarcoidosis?

A

20%

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25
Q

What is salivary glands involvement in sarcoidosis?

A

up to 50%

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26
Q

What does sarcoidosis present as?

A
  1. in young adults
  2. incidental abnormal chest x ray (no symptoms)
  3. shortness of breath, cough and abnormal chest x ray (symptomatic)
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27
Q

Young adults suffering from which 3 conditions are more likely to present with sarcoidosis?

A
  1. acute arthralgia (joint pain)
  2. erythema nodosum (inflammation of fat under skin)
  3. bilateral hilar lymphadenopathy (bilateral enlargement of nodes of pulmonary hilia)
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28
Q

Sarcoidosis usually resolves after how long in young adults?

A

Usually after 2 years

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29
Q

People who present with sarcoidosis with symptoms or without symptoms can have the condition for how long?

A

it can progress further (or resolve) but it varies

30
Q

What is the treatment for sarcoidosis?

A

Corticosteroids

31
Q

What findings need to be found to make a sarcoidosis diagnosis?

A
  • clinical findings
  • imaging findings
  • serum Ca increase
  • biopsy
  • ACE: angiotension converting enzyme; raises BP and restricts blood flow
32
Q

What histological tests are needed for a sarcoidosis diagnosis? (2)

A
  1. Kveim test (~60% positive)

2. Tuberculin test

33
Q

What is hypersensitivity pneumonitis?

A

Inflammation of alveoli due to hypersensitivity to inhaled organic dusts (sufferers usually exposed to dusts in their hobbies/occupation)

34
Q

What are the antigens which cause hypersensitivity pneumonitis? (allergens)

A
  1. thermophillic actinomycetes (farmer’s lung)
  2. bird/animal proteins (faces, bloom)
  3. fungi (aspergillus species)
  4. chemicals
    …and others
35
Q

What are 2 thermophillic actinomycetes which can cause hypersensitivity pneumonitis?

A
  1. micropolyspora faeni

2. thermoactinomyces vulgaris

36
Q

What is another name for hypersensitivity pneumonitis?

A

extrinsic allergic alveolitis

37
Q

What is an acute presentation of hypersensitivity pneumonitis?

A
  • fever/ pyrexia
  • dry cough
  • myalgia (muscle pain)
  • chills 4-9 hours after antigen exposure
  • crackles, tachypnoea and wheeze
  • precipitating antibody
38
Q

What is chronic presentation of hypersensitivity pneumonitis?

A
  • insidious (gradual but very harmful effects)
  • malaise
  • shortness of breath
  • cough
  • low grade illness
  • crackles and some wheeze (low gas tranfer)
39
Q

What can hypersensitivity pneumonitis lead to if left untreated?

A

respiratory failure

40
Q

Describe histopathology of hypersensitivity pneumonitis. (5)

A
  • immune complex mediated combined type 3 and 4 hypersensitivity reaction
  • soft centriacinar epithelioid granulomata
  • interstitial pneumonitis
  • foamy histiocytes
  • bronchiolitis obliterans
41
Q

What are type 3 and 4 hypersensitivity reactions? (which are histopathological features of hypersensitivity pneumonitis)

A
  • Type 3: immune complexes formed and deposited around body

- Type 4: Helper T cell overdrive leading to over inflammation and tissue damage

42
Q

Hypersensitivity pneumonitis affects which zone of the lungs?

A

Upper zone

43
Q

What is an example of a usual interstitial pneumonitis? (UIP)

A

Cryptogenic fibrosing alveolitis (idiopathic pulmonary fibrosis)

44
Q

Where may UIP (usual interstitial pneumonitis) be seen? aka cryptogenic fibrosing alveolitis (5)

A
  1. connective tissue diseases
    (e. g. scleroderma and rheumatoid disease)
  2. drug reaction
  3. post infection
  4. industrial exposure (asbestos)
  5. others
45
Q

What are most UIPs? (usual interstitial pneumonitis)

A

Most are CRYPTOGENIC and IDIOPATHIC (uncertain origin and cause)

46
Q

What is the histopathology of UIP (usual interstitial pneumonitis)?

A
  • patchy interstitial chronic inflammation
  • type 2 pneumocyte hyperplasia
  • smooth muscle and vascular proliferation
  • evidence of old and recent injury (temporal or spatial heterogeneity)
  • proliferation fibroblastic foci
47
Q

What group are more likely to get a Idiopathic Pulmonary Fibrosis (UIP) ? (usual interstitial pneumonitis)

A
  • elderly >50 years

- males more likely than females

48
Q

What are clinical signs of idiopathic pulmonary fibrosis? (UIP)

(5)

A
  • dyspnoea
  • cough
  • basal crackles
  • cyanosis
  • clubbing
49
Q

What is the prognosis for idiopathic pulmonary fibrosis? (UIP)

A
  • Poor prognosis
  • Most dead within 5 years
  • some fulminant (severe) and some steroid responsive
50
Q

What is seen on a chest x ray in an idiopathic pulmonary fibrosis? (UIP)

A
  • Idiopathic pulmonary fibrosis seen on basal/posterior region
  • diffuse infiltrates seen
  • cysts (looks like ground glass)
51
Q

What is the pulmonary function test indicating on in idiopathic pulmonary fibrosis?

A
  • it’s restrictive disease

- reduced gas transfer(honeycomb lung; fibrotic and cystic changes to lung, usually poor prognosis)

52
Q

What are the 6 “other patterns” of chronic response interstitial lung disease?

A
  1. NSIP (non-specific interstitial pneumonia)
  2. Silicosis
  3. COP (cryptogenic organizing pneumonia)
  4. BOOP (bronchiolitis obliterans organizing pneumonia)
  5. Smoking related fibrosis
  6. asbestos related interstitial disease
53
Q

What are the 2 types of bulk flow air movements?

A
  1. laminar (gas flows in parallel layers)
  2. turbulent (gas layers mix as they travel in different ways)
    Depends on pressure difference
54
Q

What occurs BEYOND the terminal bronchiole?

A

Diffusion (blood-air barrier)

55
Q

What is the usual affinity of haemoglobin for oxygen?

A

around 98% saturated for FlO2

56
Q

Why does CO2 rapidly equilibrates between blood and air?

A

because it’s very soluble

57
Q

What is the normal range for PaO2 in kPa?

A

10.5-13.5 kPa

58
Q

What is the normal range for PaCo2 in kPa?

A

4.8-6 kPa

59
Q

What is type 1 respiratory failure?

A

PaO2< 8kPA but PaCO2 is normal or low

o2 is low but CO2 not always

60
Q

What is type 2 respiratory failure?

A

PaCO2>6.5 kPa but PO2 is always low

o2 is low and co2 is high

61
Q

What are 4 abnormal states associated with hypoxaemia?

A
  1. alveolar hypovenilation
  2. shunt
  3. ventilation/ perfusion imbalance (V/Q)
  4. diffusion impairment
62
Q

Describe alveolar hypoventilation. (hypoxaemia)

A
  • increases PACO2 and thus increases PaCO2
  • increase in PACO2 decreases PAO2 and PaO2
  • fall in PaO2 due to hypoventilation is corrected by raising FlO2 (fraction of inspired air which is oxygen)
63
Q

What is the V/Q ratio in hypoxaemia state? (causes hypoxaemia)

A

LOW V/Q

64
Q

What causes a low V/Q ratio?

A

due to local alveolar ventilation due to some focal disease

65
Q

Do hypoxaemic patients respond well to even small increases in FlO2 while having a low V/Q ratio?

A

Yes, they respond well

66
Q

How many times faster does CO2 diffuse across alveolar membranes due to its solubility?

A

20 times faster than O2

67
Q

Do diseases impairing gas diffusion affect CO2 levels?

A
  • Usually do NOT change CO2 levels

- Diffusion impairment means it takes longer for blood and alveolar air to equilibrate particularly for O2

68
Q

How many seconds does equilibration between alveolar air and blood to mix?

A

0.25 seconds (and capillary transmit time takes 0.75 seconds)

69
Q

How can hypoxaemia be corrected?

A

By increasing FlO2 (this increases PAO2, thus increasing rate of diffusion)

70
Q

What is shunt?

A

Blood passing from right to left side of the heart WITHOUT contacting ventilated alveoli (normally 2-4% shunt)

71
Q

Pathologically, in which medical conditions does shunt happen? (3)

A
  1. congenital heart disease
  2. arterio-venous malformations
  3. pulmonary disease
72
Q

Do large shunts respond well to increase in FlO2?

A

No, they respond poorly (large shunts) because blood leaving normal lung is already 98% saturated

Systems: Resp. WK (40 decks)

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