Restrictive lung disease

Question 1

Restriction, physiological definitaion re fvc?

Answer 1

Restrictive lung disease has an FVC of less than 80% of what is expected.

Question 2

Tidal vol + Inspriatory resere and Expiratory reserve graph. What changes are seen in
Obstructive and Restrictive breathing compared to normal?

Answer 2

Obstructive = increased reserve volume and decreased expiratory reserve volume - whole graph shifts upwards.

Restrictive = reduced reserve volume, reduced vital capacity. Graph visably shrunken and lower doen.

Question 3

FEV1 marker of, FVC marker of

Answer 3

FEV1 reduced is a marker of obstructive lung disease

FVC reduced is a marker of restrictive (but also may be reduced in obstructive)

Question 4

Causes of Restrictive lung disease?

Answer 4

Lung Causes: Empysema, Sarcoidosis, Idiopathic Pulmonary Fibrosis, Hypersensitivity Pneumonitis

Pleural causes: Pleural effusion, Pneumothorax, Pleural thickening eg in Mesothelioma.

Skeletal causes: Kyphoscoliosis (curvy spine), Ankylosing Spondylitis, rib fractures, Thoracoplasty,

Muscle/Neuro Amyotrophic lateral sclerosis

Sub Diaphagmatic causes = Obestity, pregnancy,

Question 5

Where does honeycombing begin and work its way?

Answer 5

Perhipheral to central

Question 6

What colour is pleural fluid?

Answer 6

Straw coloured

Question 7

What is Thoracoplasty?

Answer 7

A surgery in the olden days to help treat TB (aerobic), by removing some ribs and deliberatly collapse chest

Question 8

Where is interstitium?

Answer 8

Between the basement membranes of epitherlium of the alveolar and the endothelium of the capillaries

Question 9

How many ILD are there? What are they and what can they result in?

Answer 9

More than 200(!) - largely due to the many types of HP.

Can result in fibrosis of the lung (Pulmonary fibrosis)

Question 10

What is sarcoidosis and histological hallmark?

Answer 10

Systemic granulomatous

Hallmark = Non-caseating granulomas

Question 11

What is erythema nodosum? What disease?

Answer 11

Sarcoidosis - red raised painful scaly patches on skin - inflammatory cells. Particularly on legs.

Erythema = red
Nodosum = bumpy

Question 12

How do you see if sarcoidosis? ie what investigation primarily?

Answer 12

CXR

Question 13

Why should sarcoid get optemetry?

Answer 13

Beacuse it can lead to anterior uveitis (inflammation at front of eye).

Can also get inflammation at the back and sides which can lead to loss of vision.

Question 14

Where do granulomas tend to locate on skin?

Answer 14

Around areas of injury, eg tattoos/scars

Question 15

Lupus Pernio - what is ?

Answer 15

Disfiguring nasal lesion (Think bluedolf - rudolf the blue nose reindeer) - rare manifestation of chronic sarcoisosis

Question 16

Who usually gets sarcoidosis?

Answer 16

Generally younger population (U40), women more often than men and worldwise

Question 17

How to investigate sarcoidosis?

Answer 17

History and exam,

CXR

Question 18

What is paratrachyl englargement and how des it present on x rays?

Answer 18

Enlargement of the lymph nodes on the sides of the trachea - present as shadowing next to the trachea, above mediastinum in lung.

Question 19

Stage 1 and 2 disease sarcoidosis (of lung)

Answer 19

So stage 1 is within lymph nodes, stage 2 is spotty lungs on XRays, so lung involvement aswell as the lymph glands

Question 20

Stage 3 Sarcoisosis of lungs

Answer 20

Larger interstitial change

Question 21

STAGE 4 sarcoisosis of lungs

Answer 21

Fibrotic change within the lungs

Question 22

How do you investigate the gland enlargement - what could be a differential diagnosis of lymph enlargement?

Answer 22

many differentials -including TB
Pulmonary function tests
Bloods/urubakysis, ECG/TB skin test/eye exam

Question 23

If in doubt, what further investigations?

Answer 23

Biopsies - bronchoscopy inc transbronchial biopsies

Question 24

Remission rates Stage 1-4?

Answer 24

Stage 1 - 55-90%
Stage 2 - 40-70%
Stage 3 - 10-20%
Stage 4- 0% - rriversiable damage has been done!

Question 25

Treatment ?

Answer 25

Not really any sypmtoms/ no vital organ involvement - stage 1 = nothing

Erythema nodosum (red node patches)/arthalgia (joint stiffness) = NSAIDS

Skin lesions/anterior uvelitis/cough - Topical steroids

Cardiac/neuro/hypercalcaemia/eye not responding to topical = systemic

Question 26

What lung complications can sarcoidosis lead to?

Answer 26

Bronchiectasis
Aspergilloma
Haemoptysys
Pneumothorax
Progressive resp. failure

Question 27

What is a fibroblastic focus and in what disease is it commonly seen?

Answer 27

swirl of fibroblasts spouting out collagen, seen in Idiopathic pulmonary fibrosis

Question 28

Who usually presents with IPF and what with? differential diagnosis?

Answer 28

Older men presenting with chronic cough and breathlessness. (can also be women - typically 60-70yo)

CRACKLES in LUNG and CLUBBING

Differential diagnosis
Infection - often thought, no improvement (inc to crackles) on antibiotics
Left Ventricular Failure

Question 29

What is traction bronchiectasis?

Answer 29

When the fibrosis pulls at the bronchioles/bronchi and makes them larger than they should be

Question 30

What are the treatment options for IPF?

Answer 30

Not really much :(
Median survival is 3 years :(
BUT some drugs are coming onto the market now which can help SLOW (not stop) the progression of fibrosis eg OAF (oral anti-fibrotic) - Pirfenidone, Nintedanib

Question 31

HP - nosey questions - why?

Answer 31

To try and figure out if they have been exposed to any organic compounds that could be causing the hypersensitivity. (Including birds, mouldy hay, malt whiskey factories and down duvets)