ILD

Question 1

What are the main ILDs?

Answer 1

Idiopathic pulmonary fibrosis
Sarcoidosis
Extrinsic allergic alveoli’s (hypersensitivity pneumonitis)

Question 2

What are ILDs?

Answer 2

Diseases affecting the interstitium

Question 3

What pattern do ILDs have on spirometry?

Answer 3

Restrictive

Question 4

What is the pathology of early stage ILD?

Answer 4

Alveolitis - injury and inflammatory cell infiltration that interferes with gas transfer

Question 5

What is the pathology of late stage ILD?

Answer 5

Fibrosis, leading to hypoxia and cardiac failure

Question 6

What is diagnosis based on?

Answer 6

Clinical failures and biopsy confirmation

Question 7

What are the two methods for biopsy, and what are the pros and cons?

Answer 7

Transbronchial

Thorascopic - more invasive but more reliable

Question 8

Which is the most common ILD?

Answer 8

Idiopathic pulmonary fibrosis

Question 9

What is idiopathic pulmonary fibrosis?

Answer 9

Progressive interstitial fibrosis of unknown cause

Question 10

What are the pathological changes in idiopathic pulmonary fibrosis?

Answer 10

Basal and sub-pleural fibrosis

Terminal lung structure replaced by dilated spaces surrounded by fibrosis

Question 11

What are the symptoms of idiopathic pulmonary fibrosis?

Answer 11

Progressive breathlessness over years

Dry cough

Question 12

What are the signs of idiopathic pulmonary fibrosis?

Answer 12

Finger clubbing

Bilateral fine inspiratory crackles

Question 13

What investigations are done for idiopathic pulmonary fibrosis - and what is seen?

Answer 13

Spirometry - restrictive
Low gas transfer
CXR - bilateral infiltrates
CT - shadowing, worse at bases and periphery, traction bronchiectasis, honeycombing

Question 14

When is a biopsy done for idiopathic pulmonary fibrosis?

Answer 14

Only if unsure after CT

Question 15

What is the treatment for idiopathic pulmonary fibrosis?

Answer 15

Oxygen if hypoxic

Lung transplant

Question 16

What is the prognosis and median survival after diagnosis for idiopathic pulmonary fibrosis?

Answer 16

Bad - 4 years

Question 17

What is sarcoidosis?

Answer 17

Multi-system granulomatous disorder of unknown cause

Question 18

What type of hypersensitivity is sarcoidosis?

Answer 18

Type 4

Question 19

What is the presentation of acute sarcoidosis?

Answer 19

Erythema nodosum
Bilateral hilar lymphadenopathy
Arthritis
Uveitis, parotitis
Fever

Question 20

What is the presentation of chronic sarcoidosis?

Answer 20

Lung infiltrates
Skin infiltrates
Peripheral lymphadenopathy
Hypercalcaemia
Other organ involvement

Question 21

What investigations are done for sarcoidosis?

Answer 21

CXR
CT
Biopsy
Spirometry
Bloods (increased calcium)

Question 22

What is seen on biopsy in sarcoidosis?

Answer 22

Non-caveating granulomas

Question 23

What is the treatment for sarcoidosis?

Answer 23

Steroids

Question 24

What is hypersensitivity pneumonitis?

Answer 24

Inflammatory condition of the small airways and interstitium with occasional granulomas caused by a trigger

Question 25

What type of hypersensitivity is hypersensitivity pneumonitis?

Answer 25

Type 3 - immune complex mediated

Question 26

What are the causes of hypersensitivity pneumonitis, and who are affected by each?

Answer 26

Thermophilic bacteria - farmers, malt workers, mushroom workers Avian proteins - bird owners Fungi Drugs - sulphasalazine

Question 27

What are the symptoms of acute hypersensitivity pneumonitis?

Answer 27

Cough Breathlessness Fever Myalgia

Question 28

When do symptoms of acute hypersensitivity pneumonitis come on?

Answer 28

Hours after exposure

Question 29

What are the signs of acute hypersensitivity pneumonitis?

Answer 29

Fever Crackles Hypoxia No wheeze

Question 30

What is seen on CXR in acute hypersensitivity pneumonitis?

Answer 30

Widespread pulmonary infiltrates

Question 31

What is the treatment for acute hypersensitivity pneumonitis?

Answer 31

Oxygen Steroids Antigen avoidance

Question 32

What are the symptoms of chronic hypersensitivity pneumonitis?

Answer 32

Breathlessness | Cough

Question 33

When do the symptoms of chronic hypersensitivity pneumonitis come on?

Answer 33

Over years

Question 34

What are the signs of chronic hypersensitivity pneumonitis?

Answer 34

Crackles

Question 35

What is seen on CXR in chronic hypersensitivity pneumonitis?

Answer 35

Pulmonary fibrosis, mostly upper zones

Question 36

How is diagnosis made in chronic hypersensitivity pneumonitis?

Answer 36

History of exposure Precipitins (IgG) Lung biopsy if unsure

Question 37

What is the treatment for chronic hypersensitivity pneumonitis?

Answer 37

Remove antigen exposure | Oral steroids if breathless or low gas transfer