Pulmonary Fibrosis

Question 1

What is pulmonary fibrosis?

Answer 1

Progressive, excessive fibrosing and connective tissue build-up in the lung interstitium

Pulmonary fibrosis affects lung function and can lead to serious respiratory issues.

Question 2

What is the typical age of onset for pulmonary fibrosis?

Answer 2

50-70 years old

Rarely affects younger individuals.

Question 3

Which gender is more commonly affected by pulmonary fibrosis?

Answer 3

Males

The condition is more prevalent in males than females.

Question 4

What characterizes familial pulmonary fibrosis?

Answer 4

2 or more blood relatives have pulmonary fibrosis

Associated with gene polymorphisms for fibrotic, inflammatory, and immune responses.

Question 5

What are the two main effects of repeated injury in pulmonary fibrosis?

Answer 5

1. Immune response releasing cytokines and growth factors
2. Activated oxidative stress causing DNA damage and apoptosis

These effects initiate fibrotic processes.

Question 6

What are the three main findings associated with the fibrotic processes in pulmonary fibrosis?

Answer 6

1. Alveolar fibrin deposition
2. Interstitium fibrin deposition
3. Honeycombing of lung parenchyma

These findings contribute to lung stiffness and breathing difficulties.

Question 7

What does scarring in pulmonary fibrosis cause?

Answer 7

Stiffness of lungs, making it harder to breathe and reducing lung volume

This can significantly impact respiratory function.

Question 8

Name one type of drug that can induce pulmonary fibrosis.

Answer 8

Immunosuppressants (e.g., bleomycin, MTX, cyclophosphamide)

Other drug categories also include antibiotics and antiarrhythmic drugs.

Question 9

What is a long-term complication of radiation therapy?

Answer 9

Radiation-induced pulmonary fibrosis

This type of fibrosis can develop due to previous radiation treatment.

Question 10

What environmental exposure is related to hypersensitivity pneumonitis?

Answer 10

Exposure to allergens such as mold and animals

An example is bird fancier’s lung.

Question 11

Identify an autoimmune condition associated with pulmonary fibrosis.

Answer 11

Sarcoidosis

Other autoimmune conditions include vasculitis and connective tissue diseases (CTDs).

Question 12

What is pneumoconiosis?

Answer 12

Inhalation of dust particles such as asbestos, coal dust, beryllium dust, silica dust

This is a type of occupational pulmonary fibrosis.

Question 13

What is the cause of idiopathic pulmonary fibrosis?

Answer 13

Unknown cause

This type of pulmonary fibrosis has no identifiable reason.

Question 14

What are the typical symptoms of pulmonary fibrosis?

Answer 14

Dyspnea worse on exertion and persistent dry cough

These symptoms are commonly reported by patients.

Question 15

What physical examination findings are associated with pulmonary fibrosis?

Answer 15

Clubbing and bilateral fine crackles near the base of lungs

These findings are indicative of alveolar concentration.

Question 16

What are the chest x-ray findings in pulmonary fibrosis?

Answer 16

Bilateral reticular opacities most prominent around lung bases, shaggy/unclear heart borders, and lung volume loss.

Question 17

What are the HRCT findings in pulmonary fibrosis?

Answer 17

Interlobular septal thickening, bilateral reticular opacities, and honeycombing (clusters of thin-walled cysts measuring 3-10 mm, most often seen in a subpleural location).

Question 18

What is included in the management of pulmonary fibrosis?

Answer 18

Pulmonary rehabilitation, ambulatory oxygen, palliative care, mechanical ventilation, and lung transplantation.

Question 19

What does pulmonary rehabilitation involve?

Answer 19

Exercise training, anxiety and depression management, nutritional counseling, and education.

Question 20

What is the target oxygen saturation for pulmonary fibrosis patients?

Answer 20

Patients aim to have an oxygen saturation of 88-92%.

Question 21

When is mechanical ventilation indicated in pulmonary fibrosis?

Answer 21

Only when clinically indicated, as it usually has poor outcomes and high risk of causing ventilator-induced lung injury.

Question 22

What is the role of lung transplantation in pulmonary fibrosis?

Answer 22

Considered in patients who don’t have any absolute contraindications.

Question 23

What are the pharmacological management options for pulmonary fibrosis?

Answer 23

Nintedanib (anti-fibrotic drug) and Pirfenidone (anti-fibrotic and anti-inflammatory drug).

Question 24

What is the use of Nintedanib?

Answer 24

It is used to slow the progression of chronic interstitial lung disease (ILD).

Question 25

What is the use of Pirfenidone?

Answer 25

It is used to slow the progression of idiopathic pulmonary fibrosis (IPF).