Interstitial Lung Disease

Question 1

What is interstitial lung disease?

Answer 1

An umbrella term to describe diseases of the lung parenchyma

Question 2

What does ILD cause aetiologically?

Answer 2

Inflammation and irreversible fibrosis

Question 3

Name 6 different ILDs.

Answer 3

• Idiopathic pulmonary fibrosis (PF)
• Drug induced PF
• Secondary PF
• Hypersensitive pneumonitis
• Cryptogenic organising pneumonia
• Asbestosis

Question 4

What is the prognosis of ILD?

Answer 4

Very poor- some as short as ~a year

Question 5

Why is ILD a restrictive pattern disease?

Answer 5

Reduces lung compliance

Question 6

What is idiopathic PF?

Answer 6

Idiopathic fibrosis of the lung parenchyma (most common ILD)

• Adults over 50
• Prognosis 2-5 years

Question 7

Which drugs cause drug-induced PF?

Answer 7

CMAN

• Cyclophosphamide
• Methotrexate
• Amiodarone
• Nitrofurantoin

Question 8

What causes secondary PF?

Answer 8

Rheumatological diseases (association with ANA primarily and RF)

• SLE
• Systemic sclerosis
• RA
• Alpha 1 anti-trypsin deficiency
• Sjogrens

Question 9

What is hypersensitivity pneumonitis?

Answer 9

Type 3 hypersensitivity reaction to an allergen

Question 10

Which allergens can cause hypersensitivity pneumonitis?

Answer 10

• Bird droppings
• Cotton
• Mould
• Mushrooms

Question 11

What is bronchoalveolar lavage?

Answer 11

Method of collecting cells from the airways:
1- Wash the airways with fluid
2- Collect that fluid back for testing

Question 12

What does bronchoalveolar lavage show in hypersensitivity pneumonitis?

Answer 12

Raised lymphocytes and mast cells

Question 13

What is cryptogenic organising pneumonia?

Answer 13

Focal area of inflammation in the lung

Question 14

What can cause cryptogenic organising pneumonia?

Answer 14

• Idiopathic
• Infection
• Inflammatory disorders
• Medications
• Radiation
• Environmental toxins and allergens

Question 15

What is asbestosis?

Answer 15

Lung fibrosis related to asbestos inhalation- asbestos is fibrogenic

Question 16

What four problems can asbestos cause?

Answer 16

• Asbestosis (PF)
• Pleural thickening and plaques
• Adenocarcinoma
• Mesothelioma

Question 17

What does PF look like on a HRCT?

Answer 17

Ground glass

Question 18

How does PF present?

Answer 18

• Dyspnoea
• Dry cough
• Fatigue

Question 19

How does cryptogenic organising pneumonia present?

Answer 19

Like pneumonia:

• Fever
• Lethargy/fatigue
• Cough
• Dyspnoea
• Chest pain

Question 20

What examination findings would you see for an ILD?

Answer 20

• BIBASAL fine end inspiratory crackles

- Clubbing

Question 21

What will lung function tests show?

Answer 21

Restrictive pattern:

• Both FVC and FEV1 less than 80%
• But ration still over 0.7

Question 22

What support should patients be offered?

Answer 22

• Advanced care planning
• Pulmonary rehabilitation
• Annual flu and 5 yearly pneumococcal vaccine

Question 23

What specific treatment for the diseases is there?

Answer 23

• Idiopathic PF- Pirfenidone (antifibrotic) or Nintendanib (mAB to tyrosine kinase)
• Hypersensitivity pneumonitis- remove allergen, give steroids
• Cryptogenic organising pneumonia- corticosteroids

Question 24

What surgery is used in some cases?

Answer 24

Transplant

Question 25

What type of respiratory failure do ILDs cause?

Answer 25

Type 2