Restrictive lung disease - clinical features and management

Question 1

What is the physiological definition of restriction?

Answer 1

A forced vital capacity <80% of the predicted value

Question 2

Working from inside to outside what should you think of when it comes to restriction?

Answer 2

lungs-> pleura->nerve or muscle-> bone and other

Question 3

Name the Main lung causes of restriction

Answer 3

interstitial lung diseases

Question 4

Name the 3 main interstitial lung diseases

Answer 4

idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis

Question 5

Name 3 pleural causes of restriction

Answer 5

pneumothorax
pleural effusion
pleural thickening

Question 6

Name 4 bone/skeletal causes of restriction

Answer 6

Kyphoscoliosis
ankylosing spondylitis
thoracoplasty
rib fractures

Question 7

Name a muscle cause of restriction

Answer 7

amyotrophic lateral sclerosis

Question 8

Name some other sub diaphragmatic causes of restriction

Answer 8

obesity

pregnancy

Question 9

Where is the interstitium?

Answer 9

Space between the epithelium of the alveolus and the endothelium of the capillary

Question 10

What are interstitial lung diseases?

Answer 10

Diseases causing thickening of the interstitium

Question 11

How many ILDs are there?

Answer 11

> 200

Question 12

What can ILDs result in?

Answer 12

pulmonary fibrosis

Question 13

What is pleural thickening related to?

Answer 13

asbestos exposure

Question 14

What is sarcoidosis?

Answer 14

multisystem granulomatous disease of unknown cause

Question 15

What is the histological hallmark of sarcoidosis?

Answer 15

non caseating granuloma

Question 16

Who does sarcoidosis most commonly affect?

Answer 16

females under 40

Question 17

Name some signs of sarcoidosis

Answer 17

red painful ankles and skin lesions - erythema nodosum
anterior uveitis
Granulomas collecting at scar tissue

Question 18

List some investigations done for sarcoidosis

Answer 18

CXR, urine samples, eye test, TB test, bloods, ECG and pulmonary function

Question 19

What is the further assessment for sarcoidosis?

Answer 19

bronchoscopy - including biopsies and ultrasound

Question 20

What surgical biopsies may be needed for sarcoidosis?

Answer 20

mediastinoscopy

video assisted thoracoscopic lung biopsy

Question 21

For mild sarcoidosis what is the treatment?

Answer 21

none

Question 22

For sarcoidosis with erythema nodosum and arthralgia what is the treatment?

Answer 22

NSAIDs

Question 23

For sarcoidosis with skin lesions and uveitis what is the treatment?

Answer 23

topical steroids

Question 24

For sarcoidosis with cardiac and neurological involvement what is the treatment?

Answer 24

systemic steroids

Question 25

About what percentage of Caucasians die from sarcoidosis?

Answer 25

<1%

Question 26

What percentage of people sustain permanent (extra) pulmonary complications due to sarcoidosis?

Answer 26

10-20%

Question 27

Name some respiratory complications sustained after sarcoidosis

Answer 27

progressive respiratory failure bronchiectasis haemoptysis and pneumothorax

Question 28

What is the ususal circumstances for IPF?

Answer 28

Males 60-70 chronic dyspnoea and cough clubbing

Question 29

What is the median survival for IPF?

Answer 29

3 years

Question 30

What are the medical options for IPF?

Answer 30

Oral anti fibrotics

Question 31

What are the surgical options for IPF?

Answer 31

transplant