Fibrosing Lung Disease

Question 1

What is idiopathic pulmonary fibrosis.

Answer 1

A type of idiopathic interstitial pneumonia.

Inflammatory cells infiltrate and pulmonary fibrosis of unknown cause.

Question 2

What does idiopathic pulmonary fibrosis usually cause.

Answer 2

It is the commonest cause of interstitial lung disease.

Question 3

What is another name for pulmonary fibrosis of unknown cause.

Answer 3

Cryptogenic fibrosis alveolitis.

Question 4

What are the symptoms of IPF. (5)

Answer 4

Dry cough. 
Exertional dyspnoea. 
Malaise. 
Weight loss. 
Arthralgia.

Question 5

What are the clinical signs of IPF. (3)

Answer 5

Cyanosis.
Finger clubbing.
Fine end inspiratory crepitations.

Question 6

What are the complications of IPF. (2)

Answer 6

Respiratory failure.

Increased risk of lung cancer.

Question 7

What is the prognosis for patients with IPF.

Answer 7

.50% 5 year survival rate. (range 1-20).

Question 8

What is interstitial lung disease.

Answer 8

A generic term used to describe a number of conditions that primarily affect the lung parenchyma in a diffuse manner

Question 9

What characterises interstitial lung disease. (2)

Answer 9

Chronic inflammation.

Progressive interstitial fibrosis.

Question 10

What are the clinical features of interstitial lung disease. (5)

Answer 10

Dyspnoea on exertion. 
Non-productive paroxysmal cough. 
Abnormal breath sounds. 
Abnormal CXR or high resolution CT. 
Restrictive pulmonary spirometry with decreased DLCO.

Question 11

What are the pathological features of interstitial lung disease. (3)

Answer 11

Fibrosis and remodelling of the interstitium.
Chronic inflammation.
Hyperplasia of type 2 epithelial cells or type 2 pneumocytes.

Question 12

What are the three broad classifications of interstitial lung disease. (3)

Answer 12

Those with a known cause.
Those associated with systemic disorders.
Idiopathic.

Question 13

What are some causes of interstitial lung disease (known cause). (5)

Answer 13

Occupational/environment (asbestosis, beryllosis, silicosis). 
Drugs (amiodarone, sulfasalazine). 
Hypersensitivity reactions (EAA). 
Infections (TB, fungi, viral). 
GORD.

Question 14

What are some causes of interstitial lung disease (systemic disorders). (4)

Answer 14

Sarcoidosis.
Rheumatoid arthritis.
SLE.
UC.

Question 15

What are some idiopathic causes of interstitial lung disease. (3)

Answer 15

Idiopathic pulmonary fibrosis.
Cryptogenic organizing pneumonia.
Lymphocytic interstitial pneumonia.

Question 16

What are some causes of upper zone shadowing on CXR. (5)

Answer 16

TB.
EAA.
Ankyloising spondylitis. 
Radiotherapy. 
Sarcoidosis, histoplasmosis.

Question 17

What are some causes of mid zone shadowing on CXR.

Answer 17

Progressive massive fibrosis (PMF).

Question 18

What are some causes of lower zone shadowing on CXR. (2)

Answer 18

Asbestosis. .

Idiopathic pulmonary fibrosis.