Restrictive Lung Disease Flashcards

1
Q

what is the main pathology behind restrictive lung disease?

A

alveolar barrier to O2 exchange

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2
Q

describe PaO2 and PaCO2 in restrictive lung disease?

A

low PaO2

normal PaCO2

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3
Q

what are the 2 types of pulmonary oedema?

A

cardiac pulmonary oedema

non cardiac pulmonary oedema

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4
Q

why does cardiac pulmonary oedema occur?

A

due to raised pulmonary pressure in the veins due to back pressure from the heart

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5
Q

why does non cardiac pulmonary oedema occur?

A

leaky pulmonary capillaries (can be caused by sepsis or trauma- ARDS)

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6
Q

what are the 3 major causes of consolidation of the alveolar air spaces?

A

infective pneuonia
infarction
others

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7
Q

what 2 types of infarction can occur in the lung and thus cause consolidation of the alveolar air?

A

pulmonary embolism

vasculitis

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8
Q

what is the name of the non-infectious type of pneumonia which causes alveolar air space consolidation?

A

cryptogenic organizing pneumonia

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9
Q

what disease is associated with cryptogenic organising pneumonia?

A

rheumatoid disease

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10
Q

what anti-arrhythmic medication is associated with cryptogenic organising pneumonia?

A

amiodarone

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11
Q

what type of hypersensitivity reaction is extrinsic allergic alveolitis?

A

type 3: immune-complex mediated

hypersensitivity pneumonitis

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12
Q

what type of hypersensitivity reaction is sarcoidosis?

A

type 4:delayed hypersensitivity

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13
Q

what 5 important signs can be caused by sarcoidosis?

A
  1. lymphadenopathy
  2. erythema nodosum
  3. uveitis
  4. myocarditis
  5. neuropathy
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14
Q

what are the 2 causes of fibrosing alveolitis?

A

rheumatoid associations

cryptogenic fibrosing alveolitis

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15
Q

what 6 important diseases cause an inflammatory infiltrate of the alveoler walls (alveolitis) and so cause restrictive disease?

A
  1. extrinsic allergic alveolitis
  2. sarcoidosis
  3. drug induced alveolitis
  4. pneumoconiosis
  5. fibrosing alveolitis
  6. autoimmune alveolitis (eg SLE)
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16
Q

what is pneumoconiosis?

A

dust-disease

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17
Q

what two types of pneumoconiosis is there?

A

fibrogenic

non fibrogenic

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18
Q

what are the 2 important types of fibrogenic pneumoconiosis?

A

asbestosis

silicosis

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19
Q

what are 3 important types of non-fibrogenic pneumoconiosis?

A

siderosis (iron)
stanosis (tin)
baritosis (barium)

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20
Q

what are the 6 important features of the clinical syndrome of restrictive lung disease?

A
  1. breathless on exertion
  2. cough (with no wheeze)
  3. finger clubbing
  4. inspiratroy lung crackles
  5. central cyanosis
  6. pulmonary fibrosis (late stage)
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21
Q

why does pulmonary fibrosis occur in late stage restrictive disease?

A

as a response to the chronic inflammation

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22
Q

what are 4 key questions you must ask to find out the cause of restrictive disease?

A

occupation?
medications?
pets?
arthritis?

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23
Q

in restrictive disease, what is the DLCO?

A

reduced

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24
Q

in restrictive disease what is the PEFR like?

A

normal

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25
Q

in restrictive disease what is FEV1, FVC and FEV1/FVC like?

A

reduced FEV1
reduced FVC
normal ratio

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26
Q

in sarcoidosis, what 3 findings would you expect when testing serum?

A

raised ACE
raised Ca
increased inflammatory markers

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27
Q

why do you use an echocardiogram in diagnosing restrictive lung disease?

A

to rule out LVF

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28
Q

how do you treat restrictive lung disease?

A
  1. remove trigger (eg dust, drug, allergen)
  2. treat any inflammation with immunosupressives
  3. give oxygen if hypoxaemia
  4. lung trasplant
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29
Q

what is the 1st line treatment for restrictive lung disease?

A

systemic corticosteroids

eg oral prednisolone

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30
Q

what is the 2nd line treatment for restrictive lung disease?

A

oral azathioprine (a steroid sparing, immunosuppressive)

31
Q

what anti-fibrotic agent can be used in some restrictive lung disease?

A

pirfenidone

32
Q

what anti-oxidant agents can be used in some restrictive lung disease?

A

acetylcysteine

33
Q

what is the type of infiltrate formed in sarcoidosis?

A

non caseating granuloma (T cell and macrophage infiltrate)

34
Q

what are the 3 types of interstitial lung disease? (in relation to timing/onset(

A
  1. acute
  2. episodic
  3. chronic (most common)
35
Q

what are the 4 types of interstitial lung disease?

A
  1. ILD of known cause
  2. idiopathic interstitial pneumonia
  3. granulomatous ILDs
  4. others
36
Q

how can sarcoidosis affect the kidneys directly?

A

granulomas in the kidneys

37
Q

how can sarcoidosis affect the kidneys indirectly?

A

due to increased calcium

38
Q

what 2 types of sarcoidosis can occur?

A

acute or chronic

39
Q

what do you see on a CXR or CT scan of the lungs in a patient with sarcoidosis?

A

bilateral hilar lymphadnopathy

peripheral nodular infiltrate

40
Q

what do you see on a tissue biopsy of a patient with sarcoidosis?

A

non-caseating granuloma

41
Q

what is the treatment of acute sarcoidosis?

A

self-limiting, usually no treatment

steroids if vital organ infected

42
Q

what is the treatment of chronic sarcoidosis?

A

oral steroids usually needed, immunosuppression (eg azathioprine, methotrexate, anti-TNF therapy)

43
Q

what do you use the raised ACE levels to measure in sarcoidosis?

A

marker of improvement

not a diagnostic test

44
Q

how long do you monitor patients for after an acute episode of sarcoidosis?

A

1 year

45
Q

what drug can cause extrinsic allergic alveiolitis?

A

belomycin (chemotherapy drug)

46
Q

when do you get symptoms of extrinsic allergic alveolitis?

A

several hours after acute exposure

47
Q

what is seen on an CXR of a patient having acute extrinsic allergic alveolitis?

A

widespread pulmonary infiltrates

48
Q

what is the treatment of acute extrinsic allergic alveolitis?

A

oxygen
steroids
antigen avoidance

49
Q

what is the cause of chronic extrinsic allergic alveolitis?

A

repeated low dose antigen exposure over time causing scarring resulting in progressive breathlessness and cough

50
Q

what would be the may clinical sign on chest examination for a patient with chronis extrinsic allergic alveolitis?

A

crackles

51
Q

what would you see in a CXR of a patient with chronic extrinsic allergic alveolitis?

A

pulmonary fibrosis (Scarring), most commonly in ther upper zones

52
Q

what is the treatment of chronic extrinsic allergic alveolitis?

A

antigen avoidance

oral steroids

53
Q

how is fibrosis shown on CXR?

A

honey-combing

54
Q

what is the most common interstitial lung disease?

A

idiopathic pulmonary fibrosis (cryptigenic fibrosins alveolitis)

55
Q

what is the pathogenesis of idiopathic pulmonary fibrosis?

A

imbalance in fibrotic repair system,

not an inflammatory disease

56
Q

what signs would you see on respiratroy examination of a patient with idiopathic pulmonary fibrosis?

A

clubbing

inspiratory crackles

57
Q

what drug is the only useful drug in idiopathic pulmonary fibrosis?

A

pirfenidone

58
Q

what is simple pneumoconiosis?

A

abnormalitiy on CXR only, no impairement of lung function

59
Q

what is complicated pneumoconiosis?

A

progressive massive fibrosis causing a restrictive pattern of lung disease
can cause SOB, haemoptysis, respiratroy failure

60
Q

what is caplans syndrome?

A

a combination of rheumatoid arthritis and pneumoconiosis that manifests as pulmonary nodules

61
Q

in simple pneumoconiosis what is seen on an CXR??

A

lots of little dots

62
Q

in complicated pneumoconiosis what is seen on a CXR?

A

balls of fibrous tissue

63
Q

what 3 categories of disease can you get from asbestos exposure?

A

pleural disease
pulmonary fibrosis
bronchial carcinoma

64
Q

what 4 types of pleural disease are related to asbestos exposure?

A
  1. benign pleural plaques
  2. acute asbestos pleuritis
  3. pleural effusion and diffuse pleural thickening
  4. malignant mesothelioma
65
Q

what are the symptoms for benign pleural plaques?

A

asymptomatic

66
Q

what are the symptoms for acute asbestos pleuritis?

A

fever, pain ,bloody pleural effusion

67
Q

what are the symptoms for pleural effusion and diffuse pleural thickening?

A

restrictive impairement

68
Q

what is the name of pulmonary fibrosis related to asbestos exposire?

A

asbestosis

a type of pneumoconiosis

69
Q

what characterises the early stage of interstitial lung disease?

A

alveolitis (injury with inflammatory cell infiltration)

70
Q

what characterises the late stage of lung disease?

A

fibrosis

71
Q

what type of lung biopsies can you take?

A
transbronchial biopsy (during bronchoscopy)
thoracoscopic biopsy (minor thoracic surgery)
72
Q

what are the pros and cons of a thoracoscopic biopsy?

A

more relieble
generates far more tissue
more invasive
(compared to a transbronchial biopsy)

73
Q

what are the 2 types of asbestos fibres?

A

serpentine (curved)

straight

74
Q

which type of asbestos is safer?

A

serpentine asbestos, gets stuck in larger airways so cant get into alveoli