Trigger 6: IPF

Question 1

incidence of IPF

Answer 1

is increasing

Question 2

idiopathic pulmonary fibrosis is

Answer 2

a type of interstitial lung disease

Question 3

pulmonary fibrosis is the ..

Answer 3

end stage of a heterogeneous group of interstitial lung disease

Question 4

which interstitial lung disease is the most common

Answer 4

IPF

Question 5

how many deaths per year in the UK

Answer 5

> 5,000

Question 6

how may patients in the EU with IPf

Answer 6

360,000

Question 7

median suvrival

Answer 7

2-3 years

Question 8

how many active IPF trial

Answer 8

> 200

Question 9

most common age to get IPF

Answer 9

70-75

Question 10

currently no treatments which

Answer 10

cure or reverse scarring

Question 11

causes of IPF

Answer 11

occurs when alveoli get damaged and increasingly scarred

Question 12

although idiopathic, IPF is linked to

Answer 12

o	dust, wood or metal exposure
o	viral infection
o	family history of IPF
o	Gastro-oesophageal reflux
disease
o	smoking

Question 13

pathological characterisation

Answer 13

• excessive accumulation of extracellular matrix (ECM) and remodelling of the lung architecture, this causes:
  1) proliferation and apoptosis of fibroblasts
  2) accumulation and breakdown of ECM- altered mesenchymal cells
  3) Loss of normal type 1 alveolar epithelium and replacement by hyperplastic type II cells found in IPF patients

Question 14

basic mechanism of IPF

Answer 14

• Injury
• Acute inflammatory responses
• persistent irritant damaging agents
• production of pro-inflammatory, angiogenic, and fibrogenic cytokines, as well as chemokines, growth factors and tissue disruptive enzymes
• increased accumulation of extracellular matrix and fibrotic lesions

Question 15

features of IPF

Answer 15

• scarring- honeycombing
• loss of elasticity
• inflammation
• loss of air filled spaces
• reduction in surface area

Question 16

thoracic imaging shows

Answer 16

pleural surface has a cobblestone appearance.

Question 17

on cross section

Answer 17

cobblestone reatino show areas of airspace enlargement and fibrotic retraction

Question 18

airspace enlargement and fibrotic retraction

Answer 18

honeycombing

Question 19

symptoms of IPF

Answer 19

• Shortness of breath
• Coughing
• Fever
• Weight loss

Question 20

IPF onset

Answer 20

rapid

Question 21

physical examination

Answer 21

• listen to the lungs

- looking at fingers

Question 22

what will the lungs sound like

Answer 22

• lunk crackles ( IPF and asbestos lungs)

- pleural rub

Question 23

what do fingers look like

Answer 23

clubbed

Question 24

which pulmonary function tests can be done

Answer 24

o	Forced vital capacity
o	Diffusion capacity of lung for CO (DLco)
o	FEV1/FVC ratio
o	Oxyhaemoglobin stas
o	6-minute walk test

Question 25

blood test

Answer 25

no specific blood test available

Question 26

why is it important that patients receive an accurate diagnosis

Answer 26

to ensure correct treatment and management decision

Question 27

diagnoses often invole

Answer 27

invasive and non invasive diagnostic procedures

Question 28

Non-invasive diagnostic procedure

Answer 28

secured by the presence of UIP (usual interstitial pneumonia)- using High resolution computed tomography (HRCT)

Question 29

what will HRCT show

Answer 29

- reticular lines | - traction bronchiectasis

Question 30

evaluation of honeycombing shown on HRCT has been found vulnerable to

Answer 30

significant inter observer variability

Question 31

diagnosis can sometime be made just using

Answer 31

high resolution computed tomography (HRCT)

Question 32

name some invasive diagnostic procedure

Answer 32

bronchoscopy bronchopleural laval (BAL) lung biopsy

Question 33

BAL is

Answer 33

analysed for infection or malignancies

Question 34

10% of patients cannot undergo surgical biopsy due to

Answer 34

AE and advanced disease

Question 35

diagnosis pathway

Answer 35

1) patient with suspected ILD 2) Comprehensive history, physical exam and appropriate lab testing 3) Thoracic imaging (Chest-xray, HRCT) 4) intermediate diagnosis

Question 36

after intermediate diagnosis what question is asked

Answer 36

is bronchoscopy likely to aid diagnosis

Question 37

is bronchoscopy likely to aid diagnosis? NO

Answer 37

surgical lung biopsy- if not excessive risk | - can make confident diagnosis

Question 38

is bronchoscopy likely to aid diagnosis? YES

Answer 38

Bronchoscopy

Question 39

Bronchospy 92)

Answer 39

- BAL | - Endoscopic lung biopsy

Question 40

BAL fluid analysis

Answer 40

- visual inspection - cell count - microbiology - malignant cell staining

Question 41

Endoscopic lung biopsy

Answer 41

- histopathology - staining - immunohistochemistry

Question 42

diffusion capacity of the lungs (DLco)

Answer 42

Diffusing capacity of the lungs for carbon monoxide (DLCO) is a medical test that determines how much oxygen travels from the alveoli of the lungs to the blood stream.

Question 43

FEV1

Answer 43

The forced expiratory volume in one second (FEV1) measurement shows the amount of air a person can forcefully exhale in one second of the FVC test. (spirometer)

Question 44

FVC

Answer 44

Vital capacity is the maximum amount of air a person can expel from the lungs after a maximum inhalation. (spirometer)

Question 45

FEV1/FVC

Answer 45

It represents the proportion of a person's vital capacity that they are able to expire in the first second of forced expiration to the full, forced vital capacity.