Restrictive Lung Disease Flashcards

1
Q

Restrictive Lung Disease

A

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

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2
Q

Intrinsic Pathology

A

Lung parenchyma

Inflammation + lung scarring

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3
Q

Intrinsic Outcome

A

Decreased alveolar:arterial gas transfer

Exudate + debris (pneuomonitis)

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4
Q

Intrinsic Disease example

A

Interstitial lung diseases

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5
Q

Extrinsic Pathology

A

Abnormal function of chest wall, pleura + neuromuscular apparatus

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6
Q

Extrinsic Outcome

A

Impaired ventilator function

Respiratory failure

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7
Q

Extrinsic Disease example

A

Myasthenia Gravis

Obesity

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8
Q

Interstitial lung disease

A

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

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9
Q

Major Interstitial lung disease

A

Idiopathic Pulmonary Fibrosis

Sarcoidosis

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10
Q

Idiopathic Pulmonary Fibrosis survival rate

A

20% at 5 years

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11
Q

Interstitial lung disease aetiology

A

1 in 3 unknown

Most ILDs rare

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12
Q

IPF Incidence + mortality

A

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

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13
Q

IPF Aetiology

A

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

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14
Q

IPF Pathophysiology

A
Environmental factors
Decreased epithelial cell integrity
Accelerated ageing-associated changes
Exaggerated fibroblast expansion
TGFbeta and FGF
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15
Q

IPF Alveolar damage

A

Dilated bronchi- traction bronchiectasis
Parenchymal Fibrosis
Alveolar remodelling
Scarred interstitium

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16
Q

IPF symptoms

A

Clubbing
Exertional dyspnoea
Dry cough

17
Q

IPF Diagnosis

A

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

18
Q

IPF Management

A

Improve symptoms + survival
Preserve Lung Functions
Decreased AEs

19
Q

IPF Medication

A

Pirfenidone

Antacid therapy

20
Q

IPF Medication DO NOT USE

A

Combo treatment: Prednisone, Azathioprine + N-acetylcysteine

21
Q

Pirfenidone

A

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

22
Q

Nintedanib

A

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

23
Q

Antacid therapy

A

IPF with Gastro-oesophageal reflux

SE- infection, cognitive function + MI

24
Q

Pulmonary rehab

A

Increases physical + psychological
Increases Walking distance
Increases quality of life

25
Non-pharmacological IPF
Pulmonary rehab Long term O2 Lung transplant
26
Long-term O2
Patients with hypoxemia at rest
27
Lung transplant
Moderate-severe disease
28
Sarcoidosis
Multi-system granulomas Immune system overdrive Most cases acute + self-limiting INFLAMMATORY
29
Sarcoidosis age
70% between 20-40
30
Sarcoidosis organs
``` Any organ Eyes 25% Lungs + lymph nodes 90% Resp 35-50% Skin 25% ```
31
Sarcoidosis pathophys
Formation of sarcoid granuloma
32
Sarcoidosis diagnosis
Bilateral hair adenopathy on chest radiograph | Lofgren syndrome
33
Sarcoidosis management
Corticosteroids