Restrictive Lung Disease

Question 1

what is the main pathology behind restrictive lung disease?

Answer 1

alveolar barrier to O2 exchange

Question 2

describe PaO2 and PaCO2 in restrictive lung disease?

Answer 2

low PaO2

normal PaCO2

Question 3

what are the 2 types of pulmonary oedema?

Answer 3

cardiac pulmonary oedema

non cardiac pulmonary oedema

Question 4

why does cardiac pulmonary oedema occur?

Answer 4

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

Question 5

why does non cardiac pulmonary oedema occur?

Answer 5

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

Question 6

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

Answer 6

infective pneuonia
infarction
others

Question 7

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

Answer 7

pulmonary embolism

vasculitis

Question 8

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

Answer 8

cryptogenic organizing pneumonia

Question 9

what disease is associated with cryptogenic organising pneumonia?

Answer 9

rheumatoid disease

Question 10

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

Answer 10

amiodarone

Question 11

what type of hypersensitivity reaction is extrinsic allergic alveolitis?

Answer 11

type 3: immune-complex mediated

hypersensitivity pneumonitis

Question 12

what type of hypersensitivity reaction is sarcoidosis?

Answer 12

type 4:delayed hypersensitivity

Question 13

what 5 important signs can be caused by sarcoidosis?

Answer 13

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

Question 14

what are the 2 causes of fibrosing alveolitis?

Answer 14

rheumatoid associations

cryptogenic fibrosing alveolitis

Question 15

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

Answer 15

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

Question 16

what is pneumoconiosis?

Answer 16

dust-disease

Question 17

what two types of pneumoconiosis is there?

Answer 17

fibrogenic

non fibrogenic

Question 18

what are the 2 important types of fibrogenic pneumoconiosis?

Answer 18

asbestosis

silicosis

Question 19

what are 3 important types of non-fibrogenic pneumoconiosis?

Answer 19

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

Question 20

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

Answer 20

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

Question 21

why does pulmonary fibrosis occur in late stage restrictive disease?

Answer 21

as a response to the chronic inflammation

Question 22

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

Answer 22

occupation?
medications?
pets?
arthritis?

Question 23

in restrictive disease, what is the DLCO?

Answer 23

reduced

Question 24

in restrictive disease what is the PEFR like?

Answer 24

normal

Question 25

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

Answer 25

reduced FEV1 reduced FVC normal ratio

Question 26

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

Answer 26

raised ACE raised Ca increased inflammatory markers

Question 27

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

Answer 27

to rule out LVF

Question 28

how do you treat restrictive lung disease?

Answer 28

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

Question 29

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

Answer 29

systemic corticosteroids | eg oral prednisolone

Question 30

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

Answer 30

oral azathioprine (a steroid sparing, immunosuppressive)

Question 31

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

Answer 31

pirfenidone

Question 32

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

Answer 32

acetylcysteine

Question 33

what is the type of infiltrate formed in sarcoidosis?

Answer 33

non caseating granuloma (T cell and macrophage infiltrate)

Question 34

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

Answer 34

1. acute 2. episodic 3. chronic (most common)

Question 35

what are the 4 types of interstitial lung disease?

Answer 35

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

Question 36

how can sarcoidosis affect the kidneys directly?

Answer 36

granulomas in the kidneys

Question 37

how can sarcoidosis affect the kidneys indirectly?

Answer 37

due to increased calcium

Question 38

what 2 types of sarcoidosis can occur?

Answer 38

acute or chronic

Question 39

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

Answer 39

bilateral hilar lymphadnopathy | peripheral nodular infiltrate

Question 40

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

Answer 40

non-caseating granuloma

Question 41

what is the treatment of acute sarcoidosis?

Answer 41

self-limiting, usually no treatment | steroids if vital organ infected

Question 42

what is the treatment of chronic sarcoidosis?

Answer 42

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

Question 43

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

Answer 43

marker of improvement | not a diagnostic test

Question 44

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

Answer 44

1 year

Question 45

what drug can cause extrinsic allergic alveiolitis?

Answer 45

belomycin (chemotherapy drug)

Question 46

when do you get symptoms of extrinsic allergic alveolitis?

Answer 46

several hours after acute exposure

Question 47

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

Answer 47

widespread pulmonary infiltrates

Question 48

what is the treatment of acute extrinsic allergic alveolitis?

Answer 48

oxygen steroids antigen avoidance

Question 49

what is the cause of chronic extrinsic allergic alveolitis?

Answer 49

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

Question 50

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

Answer 50

crackles

Question 51

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

Answer 51

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

Question 52

what is the treatment of chronic extrinsic allergic alveolitis?

Answer 52

antigen avoidance | oral steroids

Question 53

how is fibrosis shown on CXR?

Answer 53

honey-combing

Question 54

what is the most common interstitial lung disease?

Answer 54

idiopathic pulmonary fibrosis (cryptigenic fibrosins alveolitis)

Question 55

what is the pathogenesis of idiopathic pulmonary fibrosis?

Answer 55

imbalance in fibrotic repair system, | not an inflammatory disease

Question 56

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

Answer 56

clubbing | inspiratory crackles

Question 57

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

Answer 57

pirfenidone

Question 58

what is simple pneumoconiosis?

Answer 58

abnormalitiy on CXR only, no impairement of lung function

Question 59

what is complicated pneumoconiosis?

Answer 59

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

Question 60

what is caplans syndrome?

Answer 60

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

Question 61

in simple pneumoconiosis what is seen on an CXR??

Answer 61

lots of little dots

Question 62

in complicated pneumoconiosis what is seen on a CXR?

Answer 62

balls of fibrous tissue

Question 63

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

Answer 63

pleural disease pulmonary fibrosis bronchial carcinoma

Question 64

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

Answer 64

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

Question 65

what are the symptoms for benign pleural plaques?

Answer 65

asymptomatic

Question 66

what are the symptoms for acute asbestos pleuritis?

Answer 66

fever, pain ,bloody pleural effusion

Question 67

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

Answer 67

restrictive impairement

Question 68

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

Answer 68

asbestosis | a type of pneumoconiosis

Question 69

what characterises the early stage of interstitial lung disease?

Answer 69

alveolitis (injury with inflammatory cell infiltration)

Question 70

what characterises the late stage of lung disease?

Answer 70

fibrosis

Question 71

what type of lung biopsies can you take?

Answer 71

``` transbronchial biopsy (during bronchoscopy) thoracoscopic biopsy (minor thoracic surgery) ```

Question 72

what are the pros and cons of a thoracoscopic biopsy?

Answer 72

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

Question 73

what are the 2 types of asbestos fibres?

Answer 73

serpentine (curved) | straight

Question 74

which type of asbestos is safer?

Answer 74

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