Clinical Features and Management of Restrictive Lung diseases

Question 1

What is the FVC for restrictive disease?

Answer 1

<80% of normal

Question 2

What are the types of restrictive disease of the lung?

Answer 2

Interstitial lung disease
- Idiopathic pulmonary fibrosis
- Sarcoidosis
- Hypersensitivity pneumonitis
- Honeycombing fibrosis

Question 3

What are some pleural restrictive lung diseases?

Answer 3

Pleural effusions
Pneumothorax
Pleural thickening

Question 4

What are some nerve/muscle restrictive diseases?

Answer 4

Amyotrophic lateral sclerosis
Motor neurone disease

Question 5

What are some bone restrictive diseases?

Answer 5

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

Question 6

What are some other causes of restrictive lung disease?

Answer 6

Sub diaphragmatic causes
- Obesity
- Pregancy

Question 7

What are some other causes of restrictive lung disease?

Answer 7

Sub diaphragmatic causes
- Obesity
- Pregnancy

Question 8

What is the histological hallmark of sarcoidosis?

Answer 8

non-caseating granuloma

Question 9

What is the rash caused by sarcoidosis called and what does it look like?

Answer 9

Erythema nodosum
- Red
- Bumpy
- Painful
- Self limiting

Question 10

What are some other symptoms of sarcoidosis?

Answer 10

Inflammation of the eye
Skin lesions
Scalp lesions
Granulomas located around scars
Lupus pernio

Question 11

Who is more at risk of sarcoidosis?

Answer 11

Women
Young people

Question 12

How is sarcoidosis investigated? (11)

Answer 12

History
Examination
CXR
CT
Lymphadenopathy
Pulmonary function tests
Bloods
Urinalysis
ECG
TB skin test
Eye exam

Question 13

What’s a further assessment you can do for sarcoidosis?

Answer 13

Tissue diagnosis
- Bronchoscopy
- Surgical biopsies

Question 14

What is the remission rate for each stage of sarcoidosis?

Answer 14

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

Question 15

What does mild sarcoidosis look like?

Answer 15

No vital organ involvement
Normal lung function
Few symptoms

Question 16

How do you treat mild sarcoidosis?

Answer 16

No treatment - watchful waiting

Question 17

How do you treat erythema nodosum/arthralgia?

Answer 17

NSAIDs
(non steroid anti-inflammatory drugs)

Question 18

How do you treat skin lesions/anterior uveitis/cough?

Answer 18

Topical steroids

Question 19

What is treated with systemic steroids? (5)

Answer 19

Cardiac
Neurological
Eye disease not responding to topical treatment
Hypercalcaemia

Question 20

What are some complications of sarcoidosis? (5)

Answer 20

Progressive respiratory failure
Bronchiectasis
Aspergilloma
Haemoptysis
Pneumothorax

Question 21

How does IPF present?

Answer 21

Chronic breathlessness
Cough
Typically 60-70 years old
Failed Rx for LVF or infection
Clubbed fingers
Crackles that dont go away

Question 22

What would you see on a chest X-ray for IPF?

Answer 22

Shadowing at base of lung due to fibrosis

Question 23

What are the options for someone diagnosed with IPF?

Answer 23

Refer to interstitial lung disease (ILD) clinic
Medical options
Surgical options

Question 24

What are some medical options for IPF?

Answer 24

Oral anti-fibrotic (OAF)
- Pirfenidone
- Nintedanib
Palliative care

Question 25

What is the surgical option for IPF?

Answer 25

Transplant
(but most patients too old or sick)

Question 26

What are some causes of hypersensitive pneumonitis?

Answer 26

Birds
Malted whiskey
Hay
Feather bedding