Interstitial lung disease

Question 1

What is ILD?

Answer 1

Any disease process affecting the lung interstitial (alveoli, terminal bronchi)
It interferes with gas transfer

Question 2

What type of pattern do ILD’s have?

Answer 2

Restrictive lung pattern

Question 3

What are the usual symptoms of ILD?

Answer 3

Breathlessness and dry cough

Question 4

What is sarcoidosis?

Answer 4

A multisystem granulomatous (type 4 hypersensitivity) disorder, commonly affecting young adults, more common in african-caribbean women.

Question 5

what systems can be involved?

Answer 5

Its multi-system so any
Common - lung, lymph nodes, joints, liver, eyes
Uncommon - Kindeys, brain, nerves, heart

Question 6

Is it a caseating or non-caseating granulomatous disease?

Answer 6

A non caseating granuolma of unknown atieology

Question 7

Is it more or less common in smokers?

Answer 7

Less common in smokers

Question 8

What are the symptoms of sarcoidosis?

Answer 8

Erythema nodusum 
Dry cough, progressive dyspnea 
Chest pain 
Fatigue 
Weight loss
Fever
Uveitis

Question 9

What might be seen on an XRAY?

Answer 9

Bilateral Hilar lymphodenpathy 
Depending on the degree of severity you may also see....
BHL with pulmonary infiltrates
Just pulmonary infiltrates 
Fibrosis

Question 10

What would you see in a blood test in sarcoidosis?

Answer 10

ACE levels increased (activity marker not diagnostic)
Raised calcium
Increased inflammatory markers

Question 11

What might you see in a tissue biopsy of sarcoidosis?

Answer 11

Non-caseating granuloma

Question 12

What is the treatment of sarcoidosis?

Answer 12

Acute - usually self limiting

Chronic - oral steroids, immunosuppression

Question 13

What is extrinsic allergic alveolis (Hypersensitivity pneumonitis)

Answer 13

Type 3 hypersensitivity reaction. Widespread diffuse inflammatory reaction in the small airways and alveoli due to inhalation of foreigns antigens usually from animals.

Question 14

What are the causes of EAA?

Answer 14

Famers lungs 
Bird and pigeon fanciers lung 
Malt workers lung 
Mushroom workers lung 
Sugar/bagassosis workers lung

Question 15

What are the symptoms of EAA?

Answer 15

Cough, breathlessness, fever
coarse end inspiratory crackles (no wheeze)

can be acute or chronic

Question 16

When might chronic EAA occur?

Answer 16

When there has been lose dose antigen exposure over a long period of time

Question 17

What might you see in a CXR of acute and chronic EAA?

Answer 17

Acute - pulmonary infiltrates widespread

Chronic - pulmonary fibrosis (upper zones)

Question 18

What might you see in the lung function tests of acute and chronic EAA?

Answer 18

Acute - reversible restrictive

Chronic - Persistent changes

Question 19

What is the treatment for acute and chronic EAA?

Answer 19

Acute - Remove allergen, give O2, oral prednisolone.

Chronic - avoid exposure to antigen, long term oral steroids.

Question 20

What is idiopathic pulmonary fibrosis? (IPF)

Answer 20

Rare, more common in males.

Progressive decline in lung function. Inflammatory cell infiltrate and pulmonary fibrosis of unknown cause.

Most common interstitial lung disease.

Question 21

What are the symptoms of IPF?

Answer 21

Dry cough, exertional dysponea
Malaise, weight loss, joint pain
progressive breathlessness

Clubbing

Question 22

What would you hear on auscultation of IPF?

Answer 22

Bilateral fine inspiratory crackles

Question 23

What might you see on the CXR of IPF?

Answer 23

Ground glass appearance

Honeycomb lung

Question 24

What is the treatment for IPF?

Answer 24

Pirfenidone - new anti-fibrotic drug (expensive and side effects)
Oxygen if hypoxic
Lung transplant in young patients

Question 25

Describe coal workers pneumoconiosis?

Answer 25

Dust particles are retained in the small airways and alveoli of the lung for usually over 15-20 yrs.

Question 26

What are the 2 different types of syndromes you can get with coal workers lung?

Answer 26

Simple pneumoconiosis - most common, deposition of coal dust in the lungs. Associated with COPD.

Progressive massive fibrosis - due to a progression of simple. Patients develop round fibrotic masses mostly ion upper lobes. Apical destruction of the lung, resulting in emphysema and airway damage.

Question 27

What are the symptoms of coal workers pneumoconiosis?

Answer 27

cough and black sputum

Question 28

What might you find on investigation?

Answer 28

Restrictie lung function tests

Rhematoid factors and ANAs +ive.

Question 29

What is the treatment of coal workers lung?

Answer 29

Avoid further exposure

Question 30

What is silicosis?

Answer 30

Caused by an inhalation of silica particles which are very fibrogenic. Distinctive streaks of thin calcification may be seen around hilar lymph nodes (Eggshell calcification)

Question 31

What would be seen on the CXR of someone with silicosis?

Answer 31

Egg shell calcification of hilar nodes

(small numerous opacities in upper lung zones with hilar lymphadenopathy

Question 32

Describe asbestosis?

Answer 32

Fibrosis causes by asbestos dust exposure
White (chrysotile) is the least fibrogenic
Blue (Crocidolite) is the most fibrogenic

Question 33

What are some of the symptoms and signs of asbestosis?

Answer 33

Plerual plaques
Progressive breathlessness
Clubbing
Bilateral basal fine end inspiratory crackles

Question 34

What are some of the complications of asbestosis?

Answer 34

Bronchial carcinoma, Pulmonary fibrosis, adenocarcinoma…. etc