Interstitial lung disease

Question 1

What is Interstitial lung disease?

Answer 1

Umbrella term to describe conditions affecting the lung parenchyma causing inflammation and fibrosis

Question 2

What is fibrosis?

Answer 2

Replacement of normal elastic and functional lung tissue with scar tissue that is stiff and does not function effectively

Question 3

How is a diagnosis of ILD made?

Answer 3

Combination of clinical features and high resolution CT scan of the thorax

If diagnosis is unclear, biopsy can be performed

Question 4

Characteristic appearance of ILD on HRCT

Answer 4

“Ground glass” appearance

Question 5

Management of ILD

Answer 5

Generally supportive and to prevent further progression:

• Remove/ treat underlying cause
• Home O2
• Stop smoking
• Physiotherapy and pulmonary rehabilitation
• Advanced care planning and palliative care

Question 6

List 2 vaccines for patients with ILD

Answer 6

Pneumococcal and flu vaccine

Question 7

List 4 causes of ILD

Answer 7

1. Pulmonary Fibrosis
2. Hypersensitivity Pneumonitis
3. Cryptogenic Organising Pneumonia
4. Asbestosis

Question 8

List 3 ways pulmonary fibrosis can be categorised

Answer 8

1. Idiopathic
2. Drug induced
3. Secondary

Question 9

How does idiopathic Pulmonary fibrosis present?

Answer 9

Insidious onset of shortness of breath and dry cough over more than 3 months

Question 10

List 2 examination findings of Idiopathic pulmonary fibrosis

Answer 10

1. Bibasal fine inspiratory crackles
2. Finger clubbing

Question 11

List 2 medications used to slow progression of idiopathic pulmonary fibrosis

Answer 11

1. Pirfenidone: anti-fibrotic and anti-inflammatory
2. Nintedanib: monoclonal antibody targeting tyrosine kinase

Question 12

List 4 medications responsible for drug induced pulmonary fibrosis

Answer 12

• Amiodarone
• Cyclophosphamide
• Methotrexate
• Nitrofurantoin

Question 13

List 4 causes of secondary pulmonary fibrosis

Answer 13

1. Alpha-1 antitripsin deficiency
2. RA
3. SLE
4. Systemic sclerosis

Question 14

What is Hypersensitivity Pneumonitis (Extrinsic Allergic Alveolitis)

Answer 14

Type III hypersensitivity reaction to an environmental allergen that causes parenchymal inflammation and destruction

Question 15

What is a Bronchoalveolar lavage?

Answer 15

Cells collected from the airways during bronchoscopy by washing the airways with fluid then collecting that fluid for testing

Question 16

What would be seen on Bronchoalveolar lavage in Hypersensitivity Pneumonitis?

Answer 16

Raised lymphocytes and mast cells

Question 17

Management of Hypersensitivity pneumonitis?

Answer 17

1. Removing the allergen
2. Oxygen when necessary
3. Steroids

Question 18

List 4 specific causes of Hypersensitivity pneumonitis

Answer 18

1. Bird-fanciers lung: reaction to bird droppings
2. Farmers lung : reaction to mouldy spores in hay
3. Mushroom workers’ lung: reaction to specific mushroom antigens
4. Malt workers lung: reaction to mould on barley

Question 19

What is Cryptogenic Organising Pneumonia

Answer 19

Focal area of inflammation of the lung tissue

Can be idiopathic or triggered by infection, inflammatory disorders, medications, radiation or environmental toxins or allergens

Question 20

How does Cryptogenic organising pneumonia present?

Answer 20

1. SOB
2. Cough
3. Fever
4. Lethargy

Similar to infective pneumonia

Question 21

Definitive investigation of Cryptogenic organising pneumonia

Answer 21

Lung biopsy

Question 22

Treatment of Cryptogenic organising pneumonia

Answer 22

Systemic corticosteroids

Question 23

List 4 effects/consequences of Asbestos

Answer 23

1. Lung fibrosis
2. Pleural thickening and pleural plaques
3. Adenocarcinoma
4. Mesothelioma