Interstitial Lung Disease/Pulmonary Fibrosis

Question 1

Define interstitial lung disease?

Answer 1

Disorders that affect the interstitium (tissue and space around the air sacs of the lungs) causing inflammation and fibrosis

Question 2

COMMS: Pulmonary fibrosis

Answer 2

• Tissue next to lung air sacs becomes damaged
• Can occur due to number of causes
• Body tries to heal damage but leads to over-healing
• Scar formation occurs which thus damages the air sacs

Question 3

What are some causes of pulmonary fibrosis?

Answer 3

IDIOPATHIC/CRYPTOGENIC

Lung disease
-TB, Pneumonia

Autoimmune
-SLE, Scleroderma, RA

Systemic
-Vasculitis (Wegner’s, Churg-Strauss, Goodpasture’s, sarcoidosis)

Drugs
-Methotrexate, nitrofurantoin, Gold, Heroin, crack

Occupation

• Extrinsic allergic alveolitis (Farmer’s lung)
• Asbestosis, silicosis

Inherited
-Familial IPF, neurofibromatosis, tuberous sclerosis

Question 4

What are common symptoms of pulmonary fibrosis?

Answer 4

• Gradual onset SOB
• Exertional SOB
• Non productive cough
• Wheezing
• Haemoptysis
• Chest pain

Question 5

What are common signs of pulmonary fibrosis

Answer 5

• Central cyanosis
• Fine end-inspiratory pulmonary crackles
• Finger clubbing
• Pulmonary hypertension
• Right cardiac failure

Question 6

What investigations for pulmonary fibrosis?

Answer 6

Bloods

• FBC: mild anaemia
• ESR/CRP: increased
• Autoantibodies: present if autoimmune cause
• ABG: O2 desaturation

Imaging

• CXR
• High res CT

Other

• Spirometry
• Impaired gas exchange = reduced transfer factor

Question 7

What features may be seen on CXR for pulmonary fibrosis?

Answer 7

• Reticular and/or nodular opacities
• Bilateral interstitial opacities = small, irregular peripheral opacities
• Honey combing - late find

Question 8

What features are shown on spirometry of pulmonary fibrosis?

Answer 8

RESTRICTIVE PATTERN
FEV1 = reduced
FVC = reduced
FEV1/FVC = normal/increased

Question 9

What is the conservative management of pulmonary fibrosis?

Answer 9

• Smoking cessation
• Removal environmental cause
• Vaccinations
• Oxygen therapy
• Pulmonary rehabilitation

Question 10

What are the medical/surgical options for pulmonary fibrosis?

Answer 10

Medical

• immunosuppressive therapy (corticosteroids or azathioprine)
• Colchicine
• Ciclosporin
• D-penicillamine
• Lung transplantation