Interstitial Lung Disease

Question 1

What medication is most commonly used to try to prevent progression of pulmonary fibrosis?

Answer 1

High dose corticosteroids

Question 2

What is the only definitive treatment option for pulmonary fibrosis, and who could receive it?

Answer 2

Lung transplant- given to patients aged < 65 with severe, progressive disease

Question 3

What are some interventions that may help to relieve symptoms of pulmonary fibrosis?

Answer 3

Pulmonary rehabilitation and oxygen therapy

Question 4

What is the most common type of hypersensitivity pneumonitis in the UK?

Answer 4

Bird fancier’s lung

Question 5

What is the relationship between smoking and hypersensitivity pneumonitis?

Answer 5

Smoking reduces the risk of developing hypersensitivity pneumonitis

Question 6

Where in the lungs is pulmonary fibrosis caused by hypersensitivity pneumonitis most commonly found?

Answer 6

The upper lobes

Question 7

What are the three main features of interstitial lung disease?

Answer 7

Progressive breathlessness, restrictive lung function pattern, reduced transfer factor

Question 8

The diagnosis of ILD mainly depends on the use of what investigation?

Answer 8

High resolution CT

Question 9

What are the two most common interstitial lung diseases?

Answer 9

Idiopathic pulmonary fibrosis and sarcoidosis

Question 10

Name some potential causes of pulmonary fibrosis?

Answer 10

Idiopathic, connective tissue disease, drugs, radiotherapy, ARDS

Question 11

What blood test should be done to rule out connective tissue disease as a cause of pulmonary fibrosis?

Answer 11

Autoantibody screen

Question 12

What blood test should be done to rule out hypersensitivity pneumonitis as a cause of pulmonary fibrosis?

Answer 12

Avian precipitins

Question 13

What blood test should be done to rule out sarcoidosis as a cause of pulmonary fibrosis?

Answer 13

Serum ACE

Question 14

What will a high resolution CT of pulmonary fibrosis show?

Answer 14

A ground-glass appearance and honeycombing

Question 15

What further investigation can be used in those with suspected pulmonary fibrosis to exclude other diagnoses?

Answer 15

Bronchoscopy and bronchoalveolar lavage

Question 16

What are the three main areas of management for pulmonary fibrosis?

Answer 16

Treat any underlying cause, advise smoking cessation, prevent progression with medical treatment

Question 17

Give some examples of drugs which can potentially cause pulmonary fibrosis?

Answer 17

Chemotherapy agents, DMARDs and amiodarone

Question 18

Name some risk factors for the development of pulmonary fibrosis?

Answer 18

Male, increased age, smoking, occupational exposure to fumes and dusts

Question 19

What is the median age for presentation with idiopathic pulmonary fibrosis vs. pulmonary fibrosis associated with connective tissue disease?

Answer 19

70 years for idiopathic, 50-60 years for CTD

Question 20

What happens to the total lung volume in pulmonary fibrosis?

Answer 20

Decreased

Question 21

What happens to the FVC in pulmonary fibrosis?

Answer 21

Decreased

Question 22

What happens to FEV1 in pulmonary fibrosis?

Answer 22

Decreased

Question 23

What happens to the FEV1: FVC ratio in pulmonary fibrosis?

Answer 23

Normal (> 75%)

Question 24

In sarcoidosis, affected tissues are infiltrated with what?

Answer 24

Non-caseating granulomas

Question 25

Describe the typical demographic of an individual affected by sarcoidosis?

Answer 25

Female, 20-40 years, Afro-Caribbean ethnicity

Question 26

What type of hypersensitivity reaction is sarcoidosis?

Answer 26

Type IV

Question 27

Describe the four main features of acute sarcoidosis?

Answer 27

Bilateral hilar lymphadenopathy, fever, arthralgia, erythema nodosum

Question 28

What respiratory symptoms can be seen in sarcoidosis?

Answer 28

Dry cough and dyspnoea

Question 29

Eventually, progressive sarcoidosis leads to what respiratory change?

Answer 29

Pulmonary fibrosis

Question 30

Which electrolyte abnormality is often associated with sarcoidosis?

Answer 30

Hypercalcaemia

Question 31

What is the definitive investigation for diagnosing sarcoidosis?

Answer 31

Tissue biopsy

Question 32

An increased level of what can be detected in the blood of people with sarcoidosis, and correlates with disease activity?

Answer 32

Serum ACE

Question 33

Other than serum ACE, what are some other blood tests which may be increased in sarcoidosis?

Answer 33

Inflammatory markers, calcium, LFTs

Question 34

Name three features which may be seen on a CXR of sarcoidosis?

Answer 34

Hilar lymphadenopathy, pulmonary infiltrates, pulmonary fibrosis

Question 35

What imaging investigation is necessary for a diagnosis of sarcoidosis?

Answer 35

High resolution CT

Question 36

Other than sarcoidosis, what are the differentials of hilar lymphadenopathy?

Answer 36

TB and lymphoma

Question 37

Which individuals would require treatment for sarcoidosis?

Answer 37

Progressive lung disease and other end-organ involvement and hypercalcaemia

Question 38

What treatment options are available for sarcoidosis?

Answer 38

Systemic corticosteroids, hydroxychloroquine, methotrexate/infliximab

Question 39

Within what timeframe does acute sarcoidosis usually resolve?

Answer 39

2 years

Question 40

The pulmonary fibrosis associated with sarcoidosis typically affects where in the lungs?

Answer 40

Upper/mid zones

Question 41

Which type of pneumoconiosis may lead to the production of black sputum?

Answer 41

Coal worker's pneumoconiosis

Question 42

What is Caplan's syndrome?

Answer 42

Coal worker's pneumoconiosis associated with rheumatoid arthritis

Question 43

Which type of pneumoconiosis may be associated with 'eggshell' calcification on CXR?

Answer 43

Silicosis

Question 44

Having silicosis increases an individual's risk of developing which other condition?

Answer 44

Pulmonary TB

Question 45

What is pneumoconiosis?

Answer 45

Lung damage caused by inhalation of mineral dusts

Question 46

In pneumoconiosis, there is usually a history of what?

Answer 46

Occupational exposure

Question 47

Name the three most common types of pneumoconiosis seen in the UK?

Answer 47

Coal worker's pneumoconiosis, asbestosis, silicosis

Question 48

What are some factors that may help to distinguish asbestosis from idiopathic pulmonary fibrosis?

Answer 48

Younger age of onset, presence of pleural plaques and asbestos bodies

Question 49

Are there any treatment options for pneumoconiosis?

Answer 49

No

Question 50

Describe the symptoms experienced in chronic hypersensitivity pneumonitis?

Answer 50

Progressive dyspnoea and dry cough, associated with weight loss and fatigue

Question 51

What happens to the transfer factor in hypersensitivity pneumonitis?

Answer 51

Reduced

Question 52

What is the treatment for hypersensitivity pneumonitis?

Answer 52

Allergen avoidance and high dose corticosteroids

Question 53

Describe the progression of chronic hypersensitivity pneumonitis, if allergen avoidance is not adhered to?

Answer 53

Progression to end-stage pulmonary fibrosis

Question 54

What types of hypersensitivity reaction can hypersensitivity pneumonitis be caused by?

Answer 54

Type III or type IV

Question 55

How soon after exposure to the antigen does acute hypersensitivity pneumonitis occur?

Answer 55

4-6 hours

Question 56

How quickly does acute hypersensitivity pneumonitis resolve?

Answer 56

Within 48-72 hours

Question 57

Describe the symptoms of acute hypersensitivity pneumonitis?

Answer 57

Episodic breathlessness, cough, wheeze and fever

Question 58

What pattern would pulmonary function tests show in hypersensitivity pneumonitis?

Answer 58

Either a restrictive, or mixed restrictive/obstructive pattern

Question 59

Describe the typical clinical presentation of pulmonary fibrosis?

Answer 59

Progressive dyspnoea on exertion and a dry cough

Question 60

Describe what happens to O2 saturations in early stages of pulmonary fibrosis?

Answer 60

Normal at rest but decrease on exercise

Question 61

Name some clinical signs of idiopathic pulmonary fibrosis?

Answer 61

Finger clubbing, reduced chest expansion, fine, inspiratory bibasal crackles

Question 62

What investigations should be done in someone presenting with features of pulmonary fibrosis?

Answer 62

Blood tests, PFTs, CXR and high-resolution CT

Question 63

What will a CXR of idiopathic pulmonary fibrosis show?

Answer 63

Bibasal reticulonodular shadowing