Interstitial Lung Disease

Question 1

Define ILD

Answer 1

An umbrella term describing a number of conditions that affect the lung parenchyma in a diffuse manner

Question 2

Give some examples of ILD

Answer 2

Usual Interstitial Pneumonia (UIP)
Non Specific Interstitial Pneumonia (NSIP)
Cryptogenic organising pneumonia
Sarcoidosis
Extrinsic allergic alveolitis (Farmers lung, Bird fanciers lung)
Rheumatoid lung

Question 3

What patterns are shown on Pulmonary function tests in patients with ILD?

Answer 3

Restrictive

Question 4

What investigations should be done in a patient with a new diagnosis of ILD?

Answer 4

ANA
ENA
RhF
ANCA
Anti-GBM
ACE (Sarcoidosis)
IgG
HIV
Ca (Sarcoidosis)
LFTs (Sarcoidosis)
Eosinophils

Question 5

What are the classic findings of Usual Interstitial Pneumonia?

Answer 5

Clubbing
Reduced chest expansion
Fine inspiratory crackles heard in the basal areas
May have pulmonary HTN

Question 6

What is Extrinsic Alllergic Alveolitis?

Answer 6

Inhalation of an organic antigen to which the patient has been sensitised

Question 7

What is the presentation of Extrinsic Allergic Alveolitis?

Answer 7

Acute - Short period of exposure (hours), reversible and spontaneously settles within a few days
Chronic - Exposure for months-years. Harder to reverse, damage done

Question 8

What investigations would you do in a patient with suspected Sarcoidosis?

Answer 8

PFT
CXR
Bloods - Renal function, ACE, Ca
Urinary Ca
ECG
24hr tape
ECHO
Cardiac MRI
CT/MRI head - If headaches may have neur sarcoidosis

Question 9

What would you see on CT in someone with ILD?

Answer 9

Honeycombing due to the fibrosis

Beading within the fissures would occur if the patient had Sarcoidosis

Question 10

What are the treatment principles for ILD?

Answer 10

Remove exposure to cause
Stop smoking
Oxygen
MDT meeting
Palliative care
ABx if needed
Transplantation
*Can try steroids*

Question 11

What are the different causes of extrinsic allergic alveolitis?

Answer 11

Bird fancier’s lung - chronic
Farmers lung - micropolyspora faeni - acute
Malt workers lung - aspergillus

Question 12

What is the acute presentation of extrinsic allergic alveolitis?

Answer 12

4-6hrs post exposure develops fever, rigors, myalgia, dry cough, dyspnoea, crackles
NO WHEEZE

Question 13

What is the chronic presentation of extrinsic allergic alveolitis?

Answer 13

Increasing dyspnoea, weight loss, exertional dyspnoea, cor pulmonale, type I resp failure

Question 14

What is the management of acute extrinsic allergic alveolitis?

Answer 14

Remove antigen
Give O2
40mg/24hr PO Prednisolone - wean off

Question 15

What is the management of chronic extrinsic allergic alveolitis?

Answer 15

Avoid exposure to allergens or wear a facemask/+ive pressure helmet
Long term steroids
Compensation may be applicable

Question 16

What are some of the causes of fibrotic shadowing in the upper zone on CXR?

Answer 16

TB
EAA
Ankylosing spondylitis
Radiotherapy
Sarcoidosis

Question 17

What are some of the causes of fibrotic shadowing in the mid zone on CXR?

Answer 17

Progressive massive fibrosis

Question 18

What are some of the causes of fibrotic shadowing in the lower zone on CXR?

Answer 18

Usual interstitial pneumonia

Asbestosis

Question 19

What are some of the causes of bilateral hilar lymphadenopathy?

Answer 19

Sarcoidosis
Infection (TB, mycoplasma)
Malignancy - carcinoma, lymphoma
Extrinsic allergic alveolitis

Question 20

What are some of the symptoms of usual interstitial pneumonia?

Answer 20

Dry cough
Exertional dyspnoea
Malaise 
Weight loss
Arthralgia

Question 21

What are some of the signs of usual interstitial pneumonia?

Answer 21

Cyanosis
Finger clubbing
Fine end inspiratory crepetations

Question 22

What is the management of usual interstitial pneumonia?

Answer 22

Supportive therapy;
O2
Pulmonary rehabilitation
Opiates
Palliative care input
Lung transplant may need to be considered
NOT high dose steroids

Question 23

What investigations can be done for a patient with suspected usual interstitial pneumonia?

Answer 23

ABD + bloods - CRP raised, Ig raised
CXR - reduced lung volume
HRCT shows honeycombing
Restrictive pattern on spirometry
Lung biopsy may be needed for diagnosis