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8. Respiratory > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

List 3 types of interstitial lung disease.

A

Sarcoidosis
Idiopathic pulmonary fibrosis
Extrinsic allergic alveolitis (EAA)

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2
Q

Define sarcoidosis.

A

A multi-system inflammatory condition of unknown origin, mainly affecting the lungs and thoracic lymph nodes

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3
Q

Define idiopathic pulmonary fibrosis.

A

Chronic inflammation of the alveoli walls, leading to fibrosis and scarring

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4
Q

Define extrinsic allergic alveolitis (EAA).

A

An immunologically T-cell mediated inflammatory reaction in the alveoli and in the respiratory bronchioles

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5
Q

What is sarcoidosis caused by? (1)

A

Unknown

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6
Q

Describe the clinical features of sarcoidosis. (3)

A

Asymptomatic (5%)
Systemic symptoms (45%)
System specific symptoms (50%)

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7
Q

Describe the systemic symptoms of sarcoidosis. (5)

A 
Fever
Anorexia
Fatigue
Night sweats
Weight loss
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8
Q

Describe the pulmonary symptoms of sarcoidosis. (3)

A 
Dyspnoea
Cough
Discomfort
Chest pain
Wheeze
Haemoptysis (rare)
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9
Q

Apart from pulmonary symptoms, what are the other system-specific symptoms which might be seen in sarcoidosis? (4)

A

Dermatological manifestations

Ocular manifestations (e.g. uveitis)

Cardiac manifestations (e.g. left heart dysfunction)

Neurological manifestations

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10
Q

How would you diagnose sarcoidosis? (4)

A

Diagnosis of exclusion
Respiratory examination
Lung/lymph node biopsy
Chest x-ray

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11
Q

Describe the histological features of sarcoidosis found on biopsy. (2)

A

Non-necrotising granuloma formation

Asteroid bodies in giant cells

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12
Q

Describe the classification of sarcoidosis on x-ray.

HINT: there are 4 classes.

A

CLASS 1: hilar lymphadenopathy

CLASS 2: hilar lymphadenopathy WITH infiltration

CLASS 3: infiltration only

CLASS 4: bullae, fibrotic bands, bronchiectasis, or diaphragmatic tenting

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13
Q

What are 2 other names for idiopathic pulmonary fibrosis?

A

Usual interstitial pneumonia (UIP)

Cryptogenic fibrosing alveolitis (CFA)

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14
Q

What is idiopathic pulmonary fibrosis caused by? (2)

A

Unknown

Non-specific interstitial pneumonia

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15
Q

List 5 KNOWN causes of pulmonary fibrosis (i.e. not IPF).

Give examples of each.

A

Occupational/environmental, e.g.

  • Silicosis
  • Abestosis
  • EAA

Drug-induced, e.g.

  • Amiodarone
  • Nitrofurantoin
  • Methotrexate
  • Cocaine

Connective tissue disease, e.g.

  • SLE
  • RA
  • Scleroderma

Primary lung diseases, e.g.

  • Sarcoidosis
  • Lymphangioleiomyomatosis (LAM)

Other causes, e.g.

  • Genetics
  • Tumours
  • Irradiation
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16
Q

Describe the pathophysiology of idiopathic pulmonary fibrosis. (4)

A
  1. Emphysema causes loss of alveolar tissue
  2. Repetitive alveolar injury causes chronic inflammation and tissue remodelling
  3. This leads to a change in alveolar environment, e.g.
    - Oxidative stress
    - Pro-fibrotic cytokines
    - Impaired fibrinolysis
    - TIMP-MMP imbalance
    - Eicosanoid imbalance
  4. This leads to dysregulated repaid and loss of epithelial cells
    a. This causes fibrosis and scarring
17
Q

Which investigations would you do for idiopathic pulmonary fibrosis? (3)

A

High resolution CT
Video assisted thoracoscopic surgery (VATS)
Lung biopsy

18
Q

What are the histological features of idiopathic pulmonary fibrosis seen on biopsy? (5)

A 
Areas of normal and abnormal tissue
Loss of alveolar space
Fibroblastic foci
Non-necrotising granuloma formation
Bronchialisation
19
Q

How do you treat idiopathic pulmonary fibrosis? (1)

A

FGF inhibitors

20
Q

Describe the clinical features of idiopathic pulmonary fibrosis.

What are the symptoms? (5)

What are the signs O/E? (4)

A 
SYMPTOMS:
SOB
Loss of apetite
Weight loss
Fatigue
Hacking, dry cough
SIGNS ON EXAMINATION:
Bibasilar crackles
Finger clubbing
Peripheral interstitial disease pattern
Subpleural honeycomb appearance
21
Q

List 3 causes of EAA.

A

Bird fancier’s lung: bird droppings
Farmer’s lung: mouldy hay
Mushroom worker’s lung: spores, moulds

22
Q

Describe the clinical features of acute EAA. (7)

A 
Flu-like illness
Cough
High fever
Dyspnoea
Chest tightness
Malaise
Mylagia
23
Q

Describe the clinical features of chronic EAA.. (5)

A 
Dyspnoea
Sputum production
Fatigue
Anorexia
Weight loss
24
Q

Describe the signs of EAA on a CXR. (4)

A

Numerous poorly defined, small (<5mm) opacities throughout lungs
“Ground glass deformities”
Fine reticulation
Zonal distribution

25
Q

Describe the signs of EAA on a lung biopsy. (5)

A 
Non-necrotising granulomas
Bronchiolocentral distribution
Foamy macrophages in alveolar spaces
Chronic interstitial inflammation
Organising pneumonia
26
Q

Briefly describe the pathophysiology of EAA.

A
  1. Initial sensitisation of T cells
  2. Reactivation of EAA
  3. Chronic inflammation via T cells, causing:
    a. Fibrosis and interstitial scarring
    b. Emphysema
    c. Decreased oxygen absorption and hypoxaemia
    d. Airspace shadowing on CXR
27
Q

How would you treat EAA?

A
  1. Avoid triggers
  2. Corticosteroids
  3. Oxygen supplementation

8. Respiratory (14 decks)

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