Pulmonary Fibrosis

Question 1

Clinical signs

Answer 1

Central cyanosis, tachypnoea, clubbing
Signs of associated AI disease: rheumatism, SLE
Fine end-inspiratory 'velcro' crackles
Grey skin (amiodarone)
Signs of treatment: cushingoid

Question 2

Bloods for pulmonary fibrosis

Answer 2

ANA, RhF, ESR

Question 3

Changes on CXR

Answer 3

Reticulonodular shadowing
Loss of definition of heart border
Small lungs

Question 4

ABG signs

Answer 4

Type 1 respiratory failure

Question 5

Spirometry findings

Answer 5

FEV1/FVC > 0.8 (restrictive)

Question 6

Purpose of bronchoalveolar lavage

Answer 6

Done before initiating immunosuppressants to exclude infection
If lymphocytes > neutrophils indicates better prognosis (sarcoidosis)

Question 7

Specific imaging in p fibrosis

Answer 7

High resolution CT:
Bibasal subpleural honeycombing - UIP
Widespread ground glass shadowing - interstitial pneumonia typically as part of other AI
Apical - sarcoidosis, TB, ABPA, hypersensitivity pneumonitis, langerhans hystiocytosis

Question 8

Treatments for PF

Answer 8

Inflammatory e.g. Interstitial pneumonitis: immunosupression with steroids (steroids with azathioprine no longer indicated)
UIP: pirfenidone (antifibrotic)
N-acetyl cysteine (free radical scavenger)
Single lung transplant

Question 9

Unilateral fine crackles, contralateral normal breath sounds with thoracotomy scar

Answer 9

Single lung transplant patient

Question 10

Causes of basal fibrosis

Answer 10

Usual interstitial pneumonia (UIP)
Abestosis
CTD
Aspiration