Restrictive Lung Disease

Question 1

Restrictive Lung Disease

Answer 1

Reduced lung Volumes
Decreased TLC
Affects lung parenchyma, pleura, chest wall or neuromuscular apparatus

Question 2

Intrinsic Pathology

Answer 2

Lung parenchyma

Inflammation + lung scarring

Question 3

Intrinsic Outcome

Answer 3

Decreased alveolar:arterial gas transfer

Exudate + debris (pneuomonitis)

Question 4

Intrinsic Disease example

Answer 4

Interstitial lung diseases

Question 5

Extrinsic Pathology

Answer 5

Abnormal function of chest wall, pleura + neuromuscular apparatus

Question 6

Extrinsic Outcome

Answer 6

Impaired ventilator function

Respiratory failure

Question 7

Extrinsic Disease example

Answer 7

Myasthenia Gravis

Obesity

Question 8

Interstitial lung disease

Answer 8

> 300 diseases
65% Idiopathic Interstitial Pneumonias + Granulomatous DPLD
35% DPLD of known cause

Question 9

Major Interstitial lung disease

Answer 9

Idiopathic Pulmonary Fibrosis

Sarcoidosis

Question 10

Idiopathic Pulmonary Fibrosis survival rate

Answer 10

20% at 5 years

Question 11

Interstitial lung disease aetiology

Answer 11

1 in 3 unknown

Most ILDs rare

Question 12

IPF Incidence + mortality

Answer 12

Amongst most lethal ILD
About 5000 people diagnosed
1 in 3 die in 12 months

Question 13

IPF Aetiology

Answer 13

Airway remodelling –> impaired oxygenation
Progressive + incurable
Risk Factors e.g. smoking cause scarring/honeycombing in lungs –> restricts breathing + oxygen exchange

Question 14

IPF Pathophysiology

Answer 14

Environmental factors
Decreased epithelial cell integrity
Accelerated ageing-associated changes
Exaggerated fibroblast expansion
TGFbeta and FGF

Question 15

IPF Alveolar damage

Answer 15

Dilated bronchi- traction bronchiectasis
Parenchymal Fibrosis
Alveolar remodelling
Scarred interstitium

Question 16

IPF symptoms

Answer 16

Clubbing
Exertional dyspnoea
Dry cough

Question 17

IPF Diagnosis

Answer 17

Fine, high pitched bibasilar inspiratory crackles (Velcro like sound)
Medical history
Physical exam
Lung function
Chest HRCT- changes associated with traction bronchiectasis + honeycombing

Question 18

IPF Management

Answer 18

Improve symptoms + survival
Preserve Lung Functions
Decreased AEs

Question 19

IPF Medication

Answer 19

Pirfenidone

Antacid therapy

Question 20

IPF Medication DO NOT USE

Answer 20

Combo treatment: Prednisone, Azathioprine + N-acetylcysteine

Question 21

Pirfenidone

Answer 21

Anti-fibrotic agent
Decreases physiological deterioration
Increases progression free survival
SE- GI, photosensitivity, anorexia, skin rash, liver toxicity

Question 22

Nintedanib

Answer 22

Tyrosine kinase inhibitor
Decreases FVC decline
SE- diarrhoea, weight loss, liver toxicity

Question 23

Antacid therapy

Answer 23

IPF with Gastro-oesophageal reflux

SE- infection, cognitive function + MI

Question 24

Pulmonary rehab

Answer 24

Increases physical + psychological
Increases Walking distance
Increases quality of life

Question 25

Non-pharmacological IPF

Answer 25

Pulmonary rehab
Long term O2
Lung transplant

Question 26

Long-term O2

Answer 26

Patients with hypoxemia at rest

Question 27

Lung transplant

Answer 27

Moderate-severe disease

Question 28

Sarcoidosis

Answer 28

Multi-system granulomas
Immune system overdrive
Most cases acute + self-limiting
INFLAMMATORY

Question 29

Sarcoidosis age

Answer 29

70% between 20-40

Question 30

Sarcoidosis organs

Answer 30

Any organ
Eyes 25%
Lungs + lymph nodes 90%
Resp 35-50%
Skin 25%

Question 31

Sarcoidosis pathophys

Answer 31

Formation of sarcoid granuloma

Question 32

Sarcoidosis diagnosis

Answer 32

Bilateral hair adenopathy on chest radiograph

Lofgren syndrome

Question 33

Sarcoidosis management

Answer 33

Corticosteroids