Interstitial Lung Diseases (ILDs) - Idiopathic Pulmonary Fibrosis

Question 1

What is Idiopathic Pulmonary Fibrosis?

Answer 1

Progressive Fibrosis of Interstitial of Lungs with no clear cause (previously known as Cryptogenic Fibrosing Alveolitis).

Question 2

Clinical Presentation of Idiopathic Pulmonary Fibrosis.

Answer 2

1. Insidious Onset SOB - Exertion Progressive.
2. Dry Cough 3+ Months.
3. Age : 50+ in Men (2:1).
4. Bibasal Fine End-Insipiratory Crackles.
5. Finger Clubbing.

Question 3

Management of Idiopathic Pulmonary Fibrosis (4).

Answer 3

1. Pirfenidone (Antifibrotic and Anti-Inflammatory).
2. Nintedanib (Monoclonal Antibody against Tyrosine Kinase).
   * both slow progression of disease.
3. Pulmonary Rehabilitation.
4. Supplementary Oxygen and Lung Transplant.

Question 4

Investigations of Idiopathic Pulmonary Fibrosis (4).

Answer 4

1. Spirometry : Restrictive.
2. Reduced TLCO.
3. High-Resolution CT : Required for Diagnosis - Bilateral Interstitial Shadowing (small irregular peripheral opacities ‘ground-glass’ and then ‘honeycombing).
4. CXR : Bilateral Lower Zone Reticulonodular Shadowing.

Question 5

Complications of Idiopathic Pulmonary Fibrosis (4).

Answer 5

1. Type II Respiratory Failure.
2. Increased Risk of Lung Cancer.
3. Cor Pulmonale.
4. 50% Mortality in 5 Years.