Restrictive Lung Disease

Question 1

What effect does restrictive lung disease have on:
Lung compliance
Gas transfer
FEV1/FVC

Answer 1

Reduced lung compliance
Reduced gas transfer due to diffusion abnormality
Low FEV1 and FVC

Question 2

What are the 2 responses to parenchymal interstitial lung injuries?

Answer 2

Acute response

Chronic response

Question 3

What is the main example of an acute response to parenchymal interstitial lung injuries?

Answer 3

Diffuse Alveolar Damage (DAD)

Question 4

Name 3 causes of DAD?

Answer 4

Major trauma
Chemical injury
Drugs
Infection
Autoimmune disease
Radiation

Question 5

What are the 2 main responses for chronic response to parenchymal interstitial lung injuries?

Answer 5

Granulomatous responses

Usual interstitial pneumonitis

Question 6

Give 2 examples of granulmatous responses to parenchymal interstitial lung injuries?

Answer 6

Sarcoidosis

Hypersensitivity pneumonitis

Question 7

What is sarcoidosis?

Answer 7

A multi-system granulomatous disorder of unknown aetiology

Question 8

What 4 organs does sarcoidosis most usually involve?

Answer 8

Lymph nodes
Lung
Spleen
Liver

Question 9

What are farmers lung and bird fanciers lung both examples of?

Answer 9

Hypersensitivity pneumonitis

Question 10

What are 3 types of antigen that can lead to hypersensitivity pneumonitis?

Answer 10

Thermophilic actinomycetes
Animal proteins
Fungi

Question 11

Name 3 symptoms of acute presentation of hypersensitivity pneumonitis?

Answer 11

Fever
Dry cough
Crackles
Tachyopnoea
Wheeze

Question 12

Name 3 symptoms of chronic presentation of hypersensitivity pneumonitis?

Answer 12

Malaise
Dyspnoea
Cough
Crackles
Some wheeze

Question 13

What can hypersensitivity pneumonitis lead to?

Answer 13

Pulmonary fibrosis

Question 14

What is the main example of usual interstitial pneumonitis?

Answer 14

Idiopathic pulmonary fibrosis (IPF)

Question 15

What are 3 signs on examination of Idiopathic pulmonary fibrosis (IPF)?

Answer 15

Basal crackles
Cyanosis
Clubbing

Question 16

What is the physiological definition of restriction?

Answer 16

An FVC of less than 80% of normal

Question 17

Give 3 conditions originating from the lungs that cause restriction?

Answer 17

Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis

Question 18

Give 3 examples of pleural causes for restriction?

Answer 18

Pleural effusion
Pneumothorax
Pleural thickening

Question 19

Give at least 2 examples of skeletal causes of restriction?

Answer 19

Kyphoscolosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

Question 20

What is the main muscle cause of of restriction?

Answer 20

ALS (Amytrophic Lateral Sclerosis)

Question 21

What are 2 sub-diaphragmatic causes of restriction?

Answer 21

Obesity

Pregnancy

Question 22

Where is the interstitium located in the lung?

Answer 22

Between the alveolus and the capillary

Question 23

Is sarcoidosis more common in:
Men or Women?
Adults over or under 40?

Answer 23

Women

Adults under 49

Question 24

What are the 2 main investigations for sarcoidosis?

Answer 24

CXR

Pulmonary function tests

Question 25

What further treatment can be performed to identify sarcoidosis from other restrictive lung diseases?

Answer 25

Bronchoscopy (transbronchial biopsies and endobronchial ultrasound)

Question 26

What 2 medications can be given to treat sarcoidosis?

Answer 26

NSAIDs

Steroids (topical or systemic)

Question 27

What age group and gender is idiopathic pulmonary fibrosis more common in?

Answer 27

60-70 years old

Male

Question 28

What does idiopathic mean?

Answer 28

Unknown cause

Question 29

What are 2 of the drugs used to treat idiopathic pulmonary fibrosis?

Answer 29

OAF (oral antifibrotics) - pirfienidome, nintedanib

Question 30

What is the main surgical treatment of idiopathic pulmonary fibrosis?

Answer 30

Lung transplant