Interstitial Lung Disease

Question 1

d:interstial lung disease

Answer 1

Any disease process affecting lung interstitium (ie alveoli, terminal bronchi).

Question 2

symptoms of ILD

Answer 2

SOB

dry cough

Question 3

what does ILD do?

Answer 3

interferes with gas transfer

restrictive lung pattern

Question 4

what is sarcoidosis?

Answer 4

growth of tiny collections of inflammatory disease in lungs/lymph nodes but can occur anywhere

Question 5

what type of hypersensitivity reaction is sarcoidosis and is it granulomatous?

Answer 5

4 and yes

Question 6

describe the pathology of sarcoidosis

Answer 6

non-caseating granuloma

Question 7

is sarcoidosis less/more common in smokers?

Answer 7

less

Question 8

Name some symptoms of acute sarcoidosis

Answer 8

erythema nodosum
 bilateral hilar lymphadenopathy
 arthritis
 uveitis, parotitis
 fever.

Question 9

give some symptoms of chronic sarcoidosis

Answer 9

lung infiltrates (alveolitis)
	 skin infiltrations
	 peripheral lymphadenopathy
	 hypercalcaemia
	 Other organs: renal, myocardial, neurological, hepatitis, splenomegaly

Question 10

Name the tests for positive sarcoidosis and what they show

Answer 10

CXR- BHL
CT- peripheral nodular infiltrate
tissue biopsy- non-caseating granuloma
LFT- restrictive function test
BT- angiotension converting enzyme
raised calcium
increased inflammatory markers

Question 11

treatment of sarcoid

Answer 11

Steroids if vital organ affected
if chronic oral steroids normally required
immunosuppression
continuous monitoring

Question 12

d: Hypersensitivity pneumonitis (extrinsic allergic alveolitis)

Answer 12

It is an inflammation of the alveoli (airspaces) within the lung caused by hypersensitivity to inhaled organic dusts

Question 13

what type of hypersensitivity reaction is hypersensitivity pneumonitis

Answer 13

3

Question 14

what is the aetiology of HP?

Answer 14

Thermophilic actinomycetes (farmers lung, malt workers, mushroom workers), avian antigens (bird fanciers lung), drugs (gold, bleomycin, sulphasalazine

Question 15

pathology of HP

Answer 15

hypersensitivity pneumonitis

Question 16

symptoms of acute HP

Answer 16

cough, breathless, fever, myalgia –
Classically symptoms occur several hours after acute exposure (flu-like illness)
Signs: +/- pyrexia, crackles (no wheeze!), hypoxia
CxR: widespread pulmonary infiltrates

Question 17

Treatment of HP

Answer 17

oxygen, steroid and antigen avoidance

Anti-fibrotic therapy in cases of progressive fibrosis (Pirfenidone or Nintedanib)

Question 18

symptoms chronic HP ( HP II)

Answer 18

progressive breathlessness and cough

Signs: may be crackles, clubbing is unusual

Question 19

tests and what show for chronic HP?

Answer 19

CxR pulmonary fibrosis - most commonly in the upper zones
PFTs: restrictive defect (low FEV1 & FVC, high or normal ratio, low gas transfer - TLCO)

lung biopsy if in doubt.

Question 20

treatment chronic HP?

Answer 20

remove antigen exposure, oral steroids if breathless or low gas transfer.

Question 21

d: Idiopathic pulmonary fibrosis

Answer 21

chronic scarring lung disease characterized by a progressive and irreversible decline in lung function

Question 22

aetiology of IPF?

Answer 22

imbalance of fibrotic repair system

related to gastric reflux

Question 23

is IPf more/less common in smokers/

Answer 23

more

Question 24

name some secondary causes of pulmonary fibrosis

Answer 24

rheumatoid, SLE, systemic sclerosis, asbestos

- drugs - amiodarone, busulphan, bleomycin, penicillamine, nitrofurantoin, methotrexate

Question 25

symptoms IPF

Answer 25

progressive breathlessness (several years), dry cough

Question 26

examination findings IPF?

Answer 26

clubbing, bilateral fine inspiratory crackles

restrictive defect on PFT’s - reduced FEV1 and FVC with normal or raised FEV1/FVC ratio, reduced lung volumes, low gas transfer
CXR- bilateral infiltrates
CT- reticulonodular fibrotic shadowing, worse at lung bases, honeycombing

Question 27

pathology of IPF?

Answer 27

Usual Interstitial Pneumonia pattern (UIP) heterogenous fibrosis in alveolar walls with fibroblastic foci and destruction of architecture causing honeycombing. Inflammation is minimal

Question 28

treatment of IPF III

Answer 28

Treatment is frustrating.
Steroids and immunosuppressants do not change course of disease
New antifibrotic drugs have emerged in recent years – PIRFENIDONE and NINTEDANIB – only therapies to have shown in randomised controlled trials to slow down disease progression BUT very expensive, and many side-effects.
Antifibrotic therapy does not reverse fibrosis, merely slows progression

Oxygen if hypoxic.

Lung transplantation in young patients

Question 29

what is coal workers pneumonconiosis?

Answer 29

Complicated pneumoconiosis - progressive massive fibrosis  restrictive pattern with breathlessness.

Chronic bronchitis (coal dust + smoking).

Question 30

d; silicosis and how it presents

Answer 30

Simple pneumoconiosis – few symptoms;
chest X-ray abnormality (egg-shell calcification of hilar nodes).

Chronic silicosis - restrictive pattern, pulmonary fibrosis.

Question 31

pleural diseases caused by asbestos and how they present

Answer 31

1- Benign pleural plaques - asymptomatic
2- Acute asbestos pleuritis - fever, pain, bloody pleural effusion
3- Pleural Effusion and Diffuse pleural thickening - restrictive impairment
4- Malignant Mesothelioma - incurable pleural cancer. Presents with chest pain and pleural effusion. No available treatment - fatal within two years.

Question 32

name 2 other types of disease asbestos can cause?

Answer 32

Pulmonary Fibrosis - “Asbestosis” - heavy prolonged exposure. Diffuse pulmonary fibrosis and restrictive defect. Asbestos bodies in sputum. Asbestos fibres in lung biopsy.

Bronchial carcinoma - asbestos multiplies risk in smokers