Idiopathic Pulmonary Fibrosis

Question 1

Idiopathic Pulmonary Fibrosis: Define

Answer 1

Condition in which the lungs become scarred and breathing becomes increasingly difficult. It is typically seen between 50-70 yrs of age.
More common in men
Poor prognosis - average life expectancy 3-4 years.
In the young (1-20yrs) - 5-year survival rate is 50%.

Question 2

Idiopathic Pulmonary Fibrosis: Complications

Answer 2

Respiratory Failure

Increased Risk of Lung Cancer

Question 3

Idiopathic Pulmonary Fibrosis: Symptoms

Answer 3

Dry Cough 
Breathlessness - exertional
Joint Pain
Fatigue
Loss of Appetite
Weight Loss

Question 4

Idiopathic Pulmonary Fibrosis: Signs

Answer 4

Finger Clubbing
Crackles
Inspiratory Crepitations

Question 5

Idiopathic Pulmonary Fibrosis: Investigations

Answer 5

CXR - honeycomb lung if advanced, lower zone shadows
CT of Thorax - diagnostic!
Spirometry - lung expansion & FVC, lung elasticity & FEV1, both reduced leading to normal or increased FEV1/FVC ratio
Blood - FBC, ABG, CRP, ANA + in 30%
Bronchoalveolar Lavage BAL
Biopsy - diagnositic

Question 6

Idiopathic Pulmonary Fibrosis: Treatment

Answer 6

Best supportive care = Palliative, Oxygen, Pulmonary Rehab & Oxygen
Pirfenidone
Lung Transplant
Oral Anti-Fibrotic
Refer to ILD Clinic 
Nintedanib