Pulmonary Fibrosis

Question 1

What is pulomonary fibrosis?

Answer 1

Pulmonary Fibrosis describes a group of diseases which lead to interstitial lung damage and ultimately fibrosis.

Question 2

What is Idopathic pulmonary fibrosis?

Answer 2

Idiopathic pulmonary fibrosis is a progressive, fibrotic lung disease of unknown cause.

Question 3

What is the median age for Idiopathic Pulomonary Fibrosis - who is it common in?

Answer 3

The median age of presentation is 70 years old and it is more common in men and smokers.

Question 4

Presentations of idiopathic pulmonary fibrosis

Answer 4

Symptoms

Dry cough
Shortness of breath
Fatigue
Arthralgia
Signs

Cyanosis
Clubbing
Fine end-inspiratory crackles

Question 5

Complications of idiopathic pulmonary fibrosis?

Answer 5

Type 2 respiratory failure
Increased risk of lung cancer
Cor pulmonale
50% mortality in 5 years

Question 6

Investigations IPF

Answer 6

Blood tests: Check CRP, ANA, Rheumatoid Factor
Arterial blood gas: Hypoxia and hypercapnia are likely
Chest X-ray: Bilateral lower zone reticulo-nodular shadowing
High resolution CT Chest: Reticulonodular shadowing and Honeycomb lung
Spirometry: Restrictive deficit with reduced transfer factor

Question 7

Treatment of IPF

Answer 7

Conservative: Stop smoking, pulmonary rehabilitation
Medical: Long term oxygen therapy, Antifibrotic drugs such as Pirfenidine, symptom control in right sided heart failure
Surgical: Lung transplant is the only ‘cure’

Question 8

What two drugs can slow the progression?

Answer 8

Two medications are licensed that can slow the progression of the disease:

Pirfenidone is an antifibrotic and anti-inflammatory
Nintedanib is a monoclonal antibody targeting tyrosine kinase

Question 9

What drugs induce pulmonary fibrosis?

Answer 9

Amiodarone
Cyclophosphamide
Methotrexate
Nitrofurantoin

Question 10

What conditions can result in secondary pulmonary fibrosis?

Answer 10

Alpha-1 antitripsin deficiency
Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Systemic sclerosis