Station 1 - Pulmonary fibrosis

Question 1

What peripheral signs exist for pulmonary fibrosis?

What chest signs?

Answer 1

• Clubbing
• Central cyanosis
• Tachypnoea

Chest - fine end inspiratory crackles that are fixed

Question 2

What signs of autoimmune diseases should you look for in association with pulmonary fibrosis?

Answer 2

Hands:

• RA
• SLE
• Systemic sclerosis

Face:

• SLE
• Systemic sclerosis

Question 3

What signs of treatment should be looked for in assoc. with pulmonary fibrosis?

Answer 3

• Cushingoid from steroids

- Grey skin from amiodarone

Question 4

What bloods would you ask for in pulmonary fibrosis?

-what other tests

Answer 4

• ESR
• RF
• ANA

CXR/ABG/PFTs/BAL/HRCT/lung biopsy

Question 5

What CXR changes could be found in pulmonary fibrosis?

Answer 5

• reticulonodular changes
• loss of definition of either heart border
• small lungs

Question 6

What would be found on ABG in pulmonary fibrosis?

Answer 6

-T1RF

Question 7

Describe the lung functions tests in pulmonary fibrosis:

• FEV1/FVC
• TLC
• TLCo Kco

Answer 7

• FEV1/FVC >0.8 (restrictive)
• Low TLC (small lungs)
• Reduced TLCo and KCo

Question 8

What is the main indication for BAL?

Answer 8

• Exclude infection before starting immunosuppression

- if lymphocytes>neutrophils indicates a better response to steroids and better prognosis

Question 9

On HRCT - if apical fibrosis is seen, what to think of?

Answer 9

C - Coal worker's pneumoconiosis
    H - Histiocytosis/ hypersensitivity pneumonitis
    A - Ankylosing spondylitis 
    R - Radiation
    T - Tuberculosis
    S - Silicosis/sarcoidosis

Question 10

On HRCT if bibasal subpleural honeycombing is seen what is this typical of?

Answer 10

usual interstitial pnuemonia

Question 11

On HRCT if there is widespread ground glass changes what is this indicative of?

Answer 11

non-specific interstitial pneumonia (often assoc. with autoimmune causes)

Question 12

Describe some treatment modalities for pulmonary fibrosis?

Answer 12

Immunosupression
-if likely to be inflammatory i.e. non-specific interstitial pneumonia e.g. steroids

Pirfenidone
-for UIP when FEV1 50-80% predicted

N-acetyl cycsteine - free radical scavanger

Single lung transplant

Question 13

Unilater fine crackles
Thoracotomy scar
Normal breath sounds contralaterally
- diagnosis?

Answer 13

single lung transplant

Question 14

Describe the prognosis of pulmonary fibrosis?
If highly cellular with ground glass infiltrate
If honeycombing on CT
Increased risk of?

Answer 14

Very variable - dependant on aetiology

If highly cellular with ground glass infiltrate - responds to immunosuppression 80% 5 year survival

If honeycombing on CT - no response to immunosuppresion 80% 5 year mortality

Increased risk of bronchogenic carcinoma

Question 15

What are the main causes of basal fibrosis?

Answer 15

• UIP / IPF
• Asbestosis
• Connective tissue diseases
• Aspiration