Clinical Features and Management of Restrictive Lung Disease

Question 1

What is the physiological definition of restriction?

Answer 1

FVC<80% of the predicted normal

Question 2

What happens to lung volumes and capacities in restrictive lung disease?

Answer 2

All values decrease

Question 3

What is a marker of restriction?

Answer 3

Vital capacity

Question 4

What investigation is key in diagnosing restrictive lung disease?

Answer 4

Spirometry

Question 5

What are the 3 main interstitial lung diseases?

Answer 5

• Idiopathic pulmonary fibrosis
• Sarcoidosis
• Hypersensitivity pneumonitis

Question 6

What pleural causes of restrictive lung diseases are there?

Answer 6

• Pleural effusion
• Pneumothorax
• Pleural thickening

Question 7

What skeletal causes of restrictive lung disease are there?

Answer 7

• Kyphoscoliosis
• Ankylosing spondylitis
• Thoracoplasty
• Rib fractures

Question 8

What muscular causes of restrictive lung disease are there?

Answer 8

• Amyotrophic lateral sclerosis

- Many neuromuscular disorders

Question 9

What sub-diaphragmatic causes of restrictive lung disease are there?

Answer 9

• Obesity

- Pregnancy

Question 10

What is the interstitium?

Answer 10

The space between the epithelium of the alveolus and the endothelium of the capillary

Question 11

What are interstitial lung diseases?

Answer 11

More than 200 diseases that cause the thickening of the interstitium and can result in pulmonary fibrosis

Question 12

What is sarcoidosis?

Answer 12

Multisystem granulomatous disease of unknown cause

Question 13

What is the histological hallmark of sarcoidosis?

Answer 13

Non-caseating granuloma

Question 14

Who does sarcoidosis mainly affect?

Answer 14

• Adults under 40
• Women more than men
• World-wide problem

Question 15

How is sarcoidosis investigated?

Answer 15

• History
• Examination
• Chest X-ray
• Pulmonary function tests
• Bloods
• Urinalysis
• ECG
• TB skin test
• Eye exam

Question 16

How can sarcoidosis be further investigated?

Answer 16

• Bronchoscopy
• Transbronchial biopsies
• Endobronchial ultrasound

Question 17

What surgical biopsies might be carried out for sarcoidosis?

Answer 17

• Mediastinoscopy

- Video assisted thoracoscopic lung biopsy

Question 18

How does remission rate vary across the stages of sarcoidosis?

Answer 18

• Stage 1: 55-90%
• Stage 2: 40-70%
• Stage 3: 10-20%
• Stage 4: 0%

Question 19

How should mild sarcoidosis (no vital organ involvement, normal lung function, few symptoms) be treated?

Answer 19

No treatment

Question 20

How should sarcoidosis with erythema nodosum and arthralgia be treated?

Answer 20

• NSAIDs

- (Some treat with prednisolone)

Question 21

How should sarcoidosis with skin lesions, anterior uveitis and cough be treated?

Answer 21

Topical steroids

Question 22

How should sarcoidosis with cardiac, neurological, eye disease not responding to topical Rx, and/ or with hypercalcaemia be treated?

Answer 22

Large doses of systemic steroids

Question 23

What pulmonary/ extra pulmonary complications do some sustain from sarcoidosis?

Answer 23

• Progressive respiratory failure
• Bronchiectasis
• Aspergilloma
• Haemoptysis
• Pneumothorax

Question 24

What is the typical presentation for idiopathic pulmonary fibrosis?

Answer 24

-Chronic breathlessness
-Cough
Failed Rx for LVF infection
-Clubbing
-Crackles

Question 25

Who is usually affected by idiopathic pulmonary fibrosis?

Answer 25

• Commoner in men

- Aged 60-70

Question 26

What is the median survival for idiopathic pulmonary fibrosis?

Answer 26

3 years

Question 27

What options are there for management of idiopathic pulmonary fibrosis?

Answer 27

• Referral to ILD clinic
• Oral anti-fibrotic (Pirfenidone, Nintedanib)
• Palliative care
• Lung transplant in those aged under 65

Question 28

What are some causes of hypersensitivity pneumonitis?

Answer 28

• Birds

- Hay