Restrictive Lung Diseases

Question 1

What are the main types of restrictive lung diseases?

Answer 1

Sarcoidosis
Idiopathic Pulmonary Fibrosis
Hypersensitivity Pneumonitis
(These 3 = Interstitial lung diseases)

Diffuse Alveolar Damage as an acute response

Question 2

What is the definition of an interstitial lung disease?

Answer 2

Physiological definition
FVC <80%
Spirometry is key
Thickening of the interstitium.

Question 3

What are some risk factors of ILD?

Answer 3

Issues with lung, pleura, nerve/muscle, bone

Pleural effusion
PTX
Pleural thickening

Kyphoscoliosis
Ankylosing Spondylitis
Thoracoplasty
Rib fracture

ALS/MND

Obesity/pregnancy

Question 4

Who are the typical sufferers of each type of ILD?

Answer 4

Sarcoidosis
- women <40

IPF

• 60-70yo
• more common in men
• failed treatment for LVF/infection

Hypersensitivity pneumonitis
- various

Question 5

What are the symptoms/signs of ILD?

Answer 5

Reduced lung compliance
Low FEV1 and FVC but normal ratio

SOB on exertion and eventually rest
Type I respiratory failure
Heart failure

Reduced gas transfer
V/Q imbalance

Abnormal CXR

Question 6

What is the histological hallmark of sarcoidosis?

Answer 6

Non-caseating granuloma

Question 7

What investigations might be done in suspected sarcoidosis?

Answer 7

History and respiratory exam
CXR
Pulmonary function tests
Bloods, urinalysis, ECG, TB skin test, eye exam

Bronchoscopy including biopsy
Endobronchial ultrasound
Mediastinoscopy, VATS

Question 8

What is the treatment for sarcoidosis?

Answer 8

Mild with no vital organ involvement and normal lung function?
- no treatment

Erythema nodosum/arthralgia - NSAIDs

Skin lesions/anterior uveitis/cough - Topical steroids

Use systemic steroids if there are conditions not responding to topical treatments

Question 9

What is the prognosis for sarcoidosis?

Answer 9

Generally okay
Few caucasians die
10-20% sustain permanent complications

Respiratory complications can include:

• progressive respiratory failure
• bronchiectasis
• aspergilloma, haemoptysis, PTX

Question 10

What is the general presentation of someone with IPF

Answer 10

Chronic SOB
Chronic cough
Clubbing
Crackles

CXR showing diffuse infiltrates, cysts, ‘ground glass’

Question 11

What would be the treatment for someone with IPF?

Answer 11

Refer to ILD clinic
Median survival 3 years

Oral anti-fibrotic

• Pirfenidone
• Nintedanib

Palliative care
Surgical options - transplant

Question 12

What is diffuse alveolar damage?

Answer 12

Acute response to interstitial injury

Question 13

What kind of things can cause DAD?

Answer 13

Major trauma
Chemical injury/toxic inhalation
Circulatory shock
Drugs
Infection
Autoimmune disease
Radiation
Idiopathic

Question 14

What are some histological features of DAD?

Answer 14

Protein rich oedema
Fibrin
Hyaline membranes
Denuded basement membranes
Epithelial and fibroblast proliferation
Scarring - interstitium and airspace

Question 15

Examples of antigens in hypersensitivity pneumonitis?

Answer 15

Thermophilic actinomycetes

• micropolyspora faeni
• thermoactinoamyces vulgaris

Bird/animal proteins
Fungi - aspergillus
Chemicals
Others

Question 16

What is extrinsic allergic alveolitis?

Answer 16

Acute presentation of hypersensitivity pneumonitis

Question 17

Symptoms of hypersensitivity

Answer 17

Acute:
Fever
Dry cough
Myalgia
Chills
Crackles
Tachyponoea
Wheeze

Chronic:
Insidious
Malaise
SOB
Cough
Low grade illness
Crackles
Some wheeze

Question 18

What type of hypersensitivity is hypersensitivity pneumonitis?

Answer 18

Immune complex mediated combined type III and IV