Pathology of Interstitial lung disease

Question 1

Describe the early and late stage of ILD?

Answer 1

Early stage = alveolitis (injury with inflammatory cell infiltration)
Late stage = characterised by fibrosis

Question 2

What are some causes of ILD?

Answer 2

Environmental (minerals, drugs, radiation. Post-ARDS)
- Hypersensitivity (mouldy hay, avian proteins)
Unknown (idiopathic)
- Connective tissue diseases
- Fibrosing alveolitis

Question 3

What is diagnosis usually based on?

Answer 3

Clinical features often with biopsy

Question 4

What are the 2 different types of biopsy’s and describe them?

Answer 4

Transbronchial biopsy – special forceps used at bronchoscopy

Thoracoscopic biopsy – more invasive but more reliable and generates far more tissue

Question 5

Name some chronic interstitial diseases?

Answer 5

Fibrosing alveolitis
Sarcoidosis
Extrinsic allergic alveolitis (hypersensitivity pneumonitis)
Pneumoconiosis
Connective tissue diseases

Question 6

Describe fibrosing alveoli’s? (IPF)

Answer 6

aka cryptogenic fibrosing alveolitis (CFA), usual interstitial pneumonia (UIP)
Progressive interstitial fibrosis of unknown cause
Variable associated inflammation
Finger clubbing

Question 7

What is the pathology of IPF?

Answer 7

Subpleural and basal fibrosis
Inflammatory component variable
Terminally lung structure replaced by dilated spaces surrounded by fibrous walls

Question 8

Describe EAA?

Answer 8

Chronic inflammatory disease
     - Small airways
     - Interstitium
     - Occasional granulomas
Allergic origin - type 3 and 4 (CHECK)

Question 9

What are some causes of EAA?

Answer 9

Thermophilic bacteria – Farmers lung
Avian proteins – Bird fanciers lung
Fungi – Malt workers lung

Question 10

What is sarcoidosis?

Answer 10

Multisystem granulomatous disorder of unknown cause (defined by histological means)

Most cases are mild and self limiting

Question 11

What are some other manifestations of sarcoidosis?

Answer 11

Uveitis (inflammation of iris)
Erythema nodosum
Lymphadenopathy
Hypercalcaemia

Question 12

What is pneumoconiosis?

Answer 12

Lung disease caused by mineral dust exposure

E.g. asbestosis, coal workers, silicosis

Question 13

What does disease depend on?

Answer 13

Particle size (1-5μm)
Reactivity of particle
Clearance of particle
Host response

Question 14

What is common in patients with asbestos?

Answer 14

Parietal pleural plaques