Interstitial Lung Disease Flashcards

1
Q

What is sarcoidosis?

A

A multisystem inflammatory disease of unkown etiology that predominantly affects the lungs and intrathoracic lymph nodes
- Granulomas don’t have a necrotic middle (unlike TB)

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2
Q

Systemic symptoms occur in 45% of sarcoidosis cases. What are they?

A
Fever 
Anorexia
Fatigue
Night sweats 
Weight loss
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3
Q

What are the non-systemic symptoms that can arise in 50% of sarcoidosis cases?

A
Pulmonary
Dyspnoea on exertion
Cough
Chest pain
Haemoptysis (rare)
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4
Q

How is sarcoidosis identified?

A

Most cases have symptoms (systemic or not)

5% of cases are asymptomatic and are found incidentally on X-Ray

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5
Q

What body systems can sarcoidosis manifest in?

A
Dermatological
Ocular
Cardiac
Neurological
Pulmonary
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6
Q

What are the lung signs that indicate sarcoidosis?

A

Dyspnoea
Cough
Vague chest discomfort
Wheezing

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7
Q

What are the four stages of sarcoidosis (unrelated to severity or changes in pulmonary function)?

A

Stage 1 - bilateral hilar lymphadenopathy without infiltration
Stage 2 - bilateral hilar lymphadenopathy with infiltration
Stage 3 - infiltration alone
Stage 4 - fibrotic bands, bullae, hilar retraction, bronchiectasis and diaphragmatic tenting

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8
Q

How is sarcoidosis diagnosed?

A

Once one of the above stages are identified on radiograph, they are biopsied for confirmation

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9
Q

Describe the granulomas found in sarcoidosis.

A

Non-necrotising
Inflammation
Asteroid bodies inside giant cells (not specific)

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10
Q

Name some causes of pulmonary fibrosis.

A
Berylliosis
Radiation
Extrinsic Allergic Alveolitis 
Allergic Bronchopulmonary Aspergillosis 
Sarcoidosis
Tuburculosis
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11
Q

What is the most common cause of interstitial lung disease?

A

Idiopathic pulmonary fibrosis - IPF

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12
Q

What is pulmonary fibrosis?

A

Continuous fibrotic process of the lungs

  • loss of lung tissue
  • cyst formation
  • scarring of the lungs
  • accumulation of excess fibrous connective tissue
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13
Q

What is the aetiology and pathology of idiopathic pulmonay fibrosis?

A
Most common in men over the age of 50
Progressive breathlessness
Bibasilar crackles
Clubbing 
Peripheral interstitial pattern 
Subpleural honeycombing
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14
Q

What are the symptoms of IPF?

A
Breathlessness (worse with exercise)
Hacking dry cough
Fatigue and weakness (due to the inflammatory processes)
Appetitie
Weight loss
Clubbing
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15
Q

How can IPF be diagnosed?

A

High resolution CT scan

VATS - biopsy

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16
Q

What is the pathophysiology of fibrosis?

A

Trigger causes repetitive alveolar epithelial injury
This alters the alveolar microenvironment
- oxidative stress
- profibrotic cytokines
- chemokines
- eicosanoid imbalance
- impaired fibrinolysis
- TIMPs-MMPs imbalance
The cumulative effect of these cause dysregulated repair, loss of epithelial cells and accumulation of mesechymal cells
Fibrosis

17
Q

What is EAA?

A

Immunologically mediated inflammatory reaction in the alveoli and in the respiratory bronchioles
- T-cell mediated response (not atopy)
Often caused by heavy, repeated exposure to a trigger

18
Q

Name some examples of EAA?

A
Farmer's lung
Saw mill worker's lung
Bird fancier's lung
Mushroom worker's lung
Malt worker's lung
Suberosis
19
Q

What are the symptoms of EAA?

A
Flu-like illness
Cough
High fever
Chills
Dyspnoea
Chest tightness
Malaise
Myalgia
20
Q

What are the symptoms of chronic exposure EAA?

A
Dyspnoea in strain
Sputum production
Fatigue
Anorexia
Weight loss
21
Q

When does the EAA occur after exposure to the allergen?

A

4-8 hours after exposure

22
Q

What are the radiological pulmonary signs of Henoch-Schonlein purpura?

A

Numerous poorly defined small (less than 5mm) opacities throughout both lungs
Sometimes with sparing of the apices and abses
Airspace disease: usually seen as ground glass opacities
Fine reticulation may also occur
Zonal distribution
Should be equally distrusted in both lungs

23
Q

What is the pathology of Henoch-Schonlein purpura?

A
Bronchiolocentric pattern
NNGI
Foamy macrophages in alveolar spaces
Chronic interstitial inflammation
Organising pneumonia