Interstitial Lung Disease

Question 1

What is interstitial lung disease?

Answer 1

Group of conditions that cause inflammation +/or fibrosis of the lung parenchyma

Question 2

What is fibrosis?

Answer 2

Replacement of elastic, functional lung tissue with non-functional scar tissue

Question 3

Give 5 examples of interstitial lung disease

Answer 3

Idiopathic pulmonary fibrosis
Secondary pulmonary fibrosis
Hypersensitivity pneumonitis
Cryptogenic organising pneumonia
Pneumoconiosis (inc. Asbestosis)

Question 4

Give 3 symptoms of interstitial lung disease

Answer 4

SOBOE
Dry cough
Fatigue

Question 5

Describe idiopathic pulmonary fibrosis. What is the life expectancy?

Answer 5

Progressive pulmonary fibrosis with no apparent cause
Life expectancy: 2-5y

Question 6

Give 2 signs of IPF

Answer 6

Bibasal fine end-inspiratory crepitations
Clubbing

Question 7

Describe management of IPF

Answer 7

C: Pulmonary rehabilitation + stop smoking
M: Pirfenidone (reduces fibrosis + inflammation) + LTOT
S: lung transplant

Question 8

Name 4 drugs that can cause secondary pulmonary fibrosis

Answer 8

Amiodarone (+ grey/ blue skin)
Cyclophosphamide
Methotrexate
Nitrofurantoin

Question 9

Name 5 conditions that can cause secondary pulmonary fibrosis

Answer 9

Alpha-1 antitrypsin deficiency
RhA
SLE
Systemic sclerosis
Sarcoidosis

Question 10

What is hypersensitivity pneumonitis also known as?

Answer 10

Extrinsic allergic alveolitis

Question 11

What is hypersensitivity pneumonitis?

Answer 11

Inflammation of alveoli + distal bronchioles caused by an immune response to inhaled allergens

Question 12

What immune response occurs in hypersensitivity pneumonitis?

Answer 12

Type III + iV hypersensitivity

Question 13

Give 4 examples of hypersensitivity pneumonitis

Answer 13

Bird fanciers’ lung
Farmers lung
Malt workers’ lung
Mushroom workers’ lung

Question 14

List 5 symptoms in acute hypersensitivity pneumonitis

Answer 14

Cough
Dyspnoea
Fever
Malaise
Chest tightness

Question 15

List 4 ways in which chronic hypersensitivity pneumonitis manifests

Answer 15

Insidious cough/ dyspnoea
Weight loss
Clubbing
Widespread fibrotic changes mimicking IPF

Question 16

What will be heard on auscultation in hypersensitivity pneumonitis?

Answer 16

Bilateral mid-zone inspiratory crepitations

Question 17

What investigations are performed in suspected hypersensitivity pneumonitis ?

Answer 17

FBC: r/o infection
Serum precipitant antibodies
CXR
HRCT
Lung function tests
Bronchoalevolar lavage

Question 18

What may be seen on CXR in hypersensitivity pneumonitis?

Answer 18

Ground glass changes
Airspace consolidation
Interstitial opacities

Question 19

What 5 features may be seen on HRCT in hypersensitivity pneumonitis?

Answer 19

Patchy, diffuse, symmetrical ground glass opacities
Small (<5mm) centrilobular opacities
Patchy air trapping on expiratory imaging
Airspace consolidation
Changes are typically bilateral in the mid-zone

Question 20

Describe lung function tests in hypersensitivity pneumonitis

Answer 20

Acute: Restrictive pattern
Chronic: mixed pattern

TLCO reduced

Question 21

What may be found on bronchoalveolar lavage in hypersensitivity pneumonitis?

Answer 21

Antibodies to causative antigen
Lymphocytosis

Question 22

Describe first line management of hypersensitivity pneumonitis

Answer 22

Identification + removal of causative agent

Question 23

How can corticosteroids be used in hypersensitivity pneumonitis?

Answer 23

Differentiate dx: HP tends to be steroid responsive, IPF does not
Symptomatic relief

Question 24

Which patients with hypersensitivity pneumonitis may benefit from long term steroids?

Answer 24

Chronic HP may benefit from long term low-dose Prednisolone

Question 25

What are the complications of hypersensitivity pneumonitis?

Answer 25

Fibrotic + emphysematous changes following inflammation may lead to fibrosis May need LTOT Reduced ability to perform ADLs

Question 26

What is cryptogenic organising pneumonia (COP)?

Answer 26

diffuse ILD that affects distal bronchioles, respiratory bronchioles, alveolar ducts + alveolar walls

Question 27

List 4 symptoms of COP

Answer 27

Cough SOB Fever Malaise

Question 28

What may be seen on bloods in COP?

Answer 28

Leukocytosis High ESR + CRP

Question 29

What is seen on imaging in COP?

Answer 29

CXR: Bilateral patchy infiltrates, diffuse consolidative or ground glass opacities HRCT: triangle sign, air-bronchograms

Question 30

What is the distinctive triangle sign commonly seen in COP?

Answer 30

Triangular ground glass opacity with the base on the pleura + apex towards the mediastinum

Question 31

Describe lung function tests in COP

Answer 31

Classically restrictive pattern (sometimes obstructive or normal) TLCO reduced

Question 32

Describe management of COP

Answer 32

Mild: watch + wait Severe: high dose (40-60mg) Prednisolone PO

Question 33

What is asbestosis?

Answer 33

Lung fibrosis related to asbestos exposure

Question 34

What are pneumoconioses?

Answer 34

Group of lung diseases caused by inhalation of harmful dusts (aka occupational ILDs)

Question 35

Give 4 examples of pneumoconioses

Answer 35

Asbestosis Silicosis Coal workers pneumoconiosis (black lung disease) Chronic beryllium disease (Berylliosis)

Question 36

Give 2 features of silicosis on CXR

Answer 36

Progressive upper zone fibrosis "Egg shell" calcification of the hilar LNs

Question 37

Describe CXR in coal workers pneumoconiosis

Answer 37

Progressive upper zone fibrosis

Question 38

Describe CXR in berylliosis

Answer 38

Progressive upper zone linear interstitial fibrosis

Question 39

Describe lung function tests in pneumoconioses

Answer 39

May show restrictive, obstructive or normal pattern

Question 40

Describe conservative management of pneumoconioses

Answer 40

Smoking cessation Removal of occupational exposure Advice regarding compensation +/- pulmonary rehabilitation

Question 41

What medication is used in berylliosis?

Answer 41

Prednisolone 40-70mg PO