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Flashcards in Restrictive (Interstitial) Diseases Deck (75):
1

What causes outwith the lungs can cause an interstitial disease?

Skeletal, muscular or compression due to abdominal obesity or ascites

2

What muscles would have to be affected to cause interstitial lung disease?

Intercostals or diaphragm

3

What conditions can cause muscle damage leading to ILD?

Myasthenia gravis and Guillain Barre syndrome and motor neurone disease

4

What happens to physiological volumes as ILD causes chronic alveolar under-ventilation?

Decreased PaO2, increased PaCO2, decreased lung volumes

5

What can the timing of ILDs be?

Acute, episodic or chronic

6

What can internal causes of chronic IDL be generally?

Environmental or occupational agents, drugs, systemic disease, idiopathic

7

What is ILD do to disease of?

Alveolar structures

8

What are 3 of the biggest groups of causes of ILD?

Fluid in alveolar spaces, inflammatory tissues in alveolar spaces and inflammation in alveolar walls (alveolitis)

9

What can fluid in alveolar air spaces cause?

Pulmonary oedema or non-cardiac pulmonary oedema

10

What causes pulmonary oedema?

Increased pulmonary venous pressure

11

What causes non-cardiac pulmonary oedema?

Normal pulmonary venous pressure but leaky capillaries due to sepsis or trauma

12

What can inflammatory components in alveolar spaces cause? Give examples.

Consolidation (infective pneumonia), infarction (PE, vasculitis)

13

What can cause alveolitis?

Granulomatous, extrinsic allergic (i.e. from birds), sarcoidosis, drugs, fibrosing, autoimmune

14

What is sarcoidosis?

Multi system disorder causing lymphadenopathy

15

What are other causes of ILD?

Rheumatoid arthritis, drugs, idiopathic

16

What is an example of something causing eosinophilic alveolitis?

Aspergillus

17

What do clinical features of ILD include?

Cough but no wheeze, breathless on exertion, finger clubbing, inspiratory crackles, central cyanosis

18

What can later stage ILD cause?

Pulmonary fibrosis

19

What can help make a diagnosis of ILD?

History, decreased lung volumes but normal peak flow, low SpO2, CXR, ECHO, HRCT

20

What will a CXR of ILD show?

Bilateral diffuse alveolar infiltrates

21

Why is an ECHO done for ILD?

To exclude heart failure

22

What is the treatment for ILD?

Remove trigger and treat inflammation: 1st line systemic corticosteroids 2nd line azathioprine. Give oxygen if hypoxic.

23

What is the last resort treatment for ILD?

Lung transplant

24

What is another name for extrinsic allergic alveolitis?

Hypersensitivity pneumonitis

25

What are examples of extrinsic allergic alveolitis?

Bird fancier's lung, farmer's lung

26

How would you describe a chronic case of extrinsic allergic alveolitis?

Extensive fibrosis with honeycombing and air-trapping

27

What are examples of pneumoconiosis?

Asbestosis, silicosis, coal worker's lung

28

What type of lung disease is asbestosis?

Fibrotic

29

What colours can asbestos be and which is the worst?

Blue, brown, white- blue is worst

30

What is mesothelioma and when can it occur?

Malignant thickening of the pleura which can occur 20-40 years after asbestos exposure

31

Asbestos exposure combined with what makes a diagnosis even worse?

Smoking

32

What are some effects of traffic fumes?

Reduced lung growth in children and increased rates of asthma and COPD

33

What effects can many inhaled substances also have outside the lung?

Low birth weight, appendicitis, stroke, neurological outcomes

34

What is sarcoidosis?

Multi-system granulomatous type IV hypersensitivity of unknown cause

35

What type of granulomas are involved in sarcoidosis?

Non-caseating

36

Who is sarcoidosis less common in?

Smokers

37

What will acute sarcoidosis cause?

Erythema nodosum, bilateral hilar lymphadenopathy, arthritis and fever

38

What does chronic sarcoidosis cause?

Lung and skin infiltrates, peripheral lymphadenopathy and hypercalcaemia

39

What are some differentials of sarcoidosis?

TB, lymphoma, carcinoma, fungal infection

40

How is sarcoidosis diagnosed?

CXR, tissue biopsy, pulmonary function tests, bloods

41

What will a CXR of sarcoidosis cause?

Peripheral nodular infiltrate

42

What will a tissue biopsy of sarcoidosis show?

Non-caseating granulomas

43

What will pulmonary function tests for sarcoidosis show?

Restrictive pattern

44

What will blood tests for sarcoidosis show?

Increased calcium and inflammatory markers

45

What is the treatment for acute sarcoidosis?

Self limiting

46

What is the treatment for chronic sarcoidosis?

Oral steroids or immunosuppression if a major organ is involved

47

What is extrinsic allergic alveolitis/hypersensitivity pneumonitis?

Type III reaction to antigen lymphocyte alveolitis

48

How will acute extrinsic allergic alveolitis present?

A few hours after antigen exposure with dry cough, SOB, myalgia and fever

49

What signs may be present in acute extrinsic allergic alveolitis?

Crackles but no wheeze, hypoxia

50

How do you treat acute extrinsic allergic alveolitis?

Oxygen, steroids and antigen avoidance

51

What does chronic extrinsic allergic alveolitis cause?

Dry cough, progressive breathlessness, crackles

52

Where is the fibrosis most common in extrinsic allergic alveolitis?

Upper zones

53

What may be increased in extrinsic allergic alveolitis?

IgG

54

How do you treat chronic extrinsic allergic alveolitis?

Allergen avoidance and maybe oral steroids

55

Though no direct cause is known, what are some secondary causes of idiopathic pulmonary fibrosis?

Smoking, rheumatoid, SLE, sclerosis, drugs, asbestos

56

How does idiopathic pulmonary fibrosis present?

Progressive breathlessness and dry cough, clubbing and bibasal fine inspiratory crackles with decreased lung volumes

57

What will a CT of idiopathic pulmonary fibrosis show?

Reticulonodular shadowing worse at lung bases an peripheries

58

What is the buzzword associated with idiopathic pulmonary fibrosis?

Ground glass appearance on CXR

59

What are some differentials of idiopathic pulmonary fibrosis?

Occupational or connective tissue disease, LVF, extrinsic allergic alveolitis or sarcoidosis

60

What is the treatment for idiopathic pulmonary fibrosis?

No treatment really- don't give steroids or immunosuppressants. Can give oxygen if hypoxic or lung transplant if patient is young

61

What is the prognosis for idiopathic pulmonary fibrosis?

Most progress to respiratory failure and live for 4/5 years after diagnosis

62

What are normal findings of coal worker's pneumoconiosis?

CXR abnormality with no impairment of lung function

63

What will complicated pneumoconiosis show?

Massive fibrosis

64

What is Caplan's syndrome and what will it show?

Rheumatoid pneumoconiosis- pulmonary nodules

65

What will show 'egg shell calcification of hilar nodes' on CXR?

Silicosis

66

What are the symptoms of benign pleural plaques?

Asymptomatic

67

What are symptoms of acute asbestos pleuritic?

Fever, pain and bloody pleural effusion

68

What will mesothelioma present with and what is the treatment?

Chest pain and pleural effusion- no treatment

69

What are type 1 alveolar cells?

Squamous epithelial cells which line most of the alveolar surface

70

What are type 2 alveolar cells?

Squamous epithelial cells which make up the rest of the alveolar surface but are also secretory cells

71

What exemplifies early stage acute ILD?

Adult respiratory distress syndrome

72

What is fibrosis get alveolitis?

Subpleural and basal fibrosis with variable inflammatory components

73

Are connective tissue diseases milder or harsher than fibrosis alveolitis?

Milder

74

What can often accompany connective tissue diseases?

Pleural effusions and rheumatoid nodules

75

What 4 things can asbestos cause?

Pleural plaques, interstitial fibrosis (asbestosis), bronchial carcinoma, mesothelioma