Restrictive lung disease

Question 1

What is the physiological definition of restrictive lung disease?

Answer 1

Disease of the lung causing FVC to be <80% of the predicted normal of their age/sex/ethnicity etc

Question 2

What can cause restriction of the lungs?

Answer 2

Any disease that affects:

• lungs
• pleura
• nerves or muscles of the thorax
• bones

Question 3

List some lung diseases that can cause restrictive lung disease

Answer 3

Interstitial lung diseases such as

• idiopathic pulmonary fibrosis
• sarcoidosis
• hypersensitivity pneumonitis

These all lead to fibrosis and honeycombing of the lung tissue

Question 4

List some skeletal causes of restrictive lung disease

Answer 4

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty - removal of ribs for TB
Rib fractures

Question 5

List some muscular causes of restrictive lung disease

Answer 5

Amyotrophic lateral sclerosis (ALS, MND)

Obesity/Pregnancy - can affect diaphragm movement

Question 6

Describe sarcoidosis

Answer 6

Multisystem granulomatous disease of unknown cause, leading to the development of non-caveating granulomas, caused by an inappropriate immune response to an environmental, occupational or infectious agent

Question 7

Describe the endocrinology typical of sarcoidosis

Answer 7

High blood calcium
Normal PTH
Increased ACE in blood

Question 8

List some common presentations of sarcoidosis

Answer 8

Erythema nodosum - tender red bumps
Lupus pernio - raised hard lesions
Painful joints
Eye problems - uveitis, uveoparotitis, retinal inflammation

Question 9

In what group of people is sarcoidosis usually seen in?

Answer 9

Women >40yo, slightly increased prevalence in Northern Europeans and Afro-caribeans

Question 10

Describe some clinical signs of sarcoidosis

Answer 10

Bronchoscopy - white patches
Blood tests - PTH, calcium, ACE
Positive tissue biopsy sample

Question 11

How do you treat sarcoidosis?

Answer 11

No symptoms - nothing

Erythema nodosum - NSAIDs

More severe - steroids (topical or systemic)

Drugs only half the rate of progression

Question 12

What % of those with sarcoidosis develop permanent pulmonary or extra-pulmonary complications?

Answer 12

10-20%

Question 13

List some respiratory complications of sarcoidosis

Answer 13

Progressive respiratory failure
Bronchiectasis
Aspergilloma
Haemoptysis
Pneumothorax

Question 14

Describe the signs and symptoms of sarcoidosis

Answer 14

Chronic SOB and cough
Typically older
No signs of infection or LVF
Clubbing of fingers
Crackles in lungs

Question 15

When is surgery offered to those with sarcoidosis?

Answer 15

Patients <65 yo

Question 16

Describe hypersensitivity pneumonitis

Answer 16

Inflammation of the alveoli due to hypersensitivity to inhaled organic dusts.

Question 17

What agents are linked to the development of hypersensitivity pneumonitis?

Answer 17

Budgie dust and pigeon fanciers

Farmers and hay bales

Question 18

What type of hypersensitivity reactions are involved in hypersensitivity pneumonitis?

Answer 18

Type III and IV HS

Question 19

How do you treat hypersensitivity pneumonitis?

Answer 19

Remove trigger e.g. budgie

Corticosteroids e.g. prednisolone