Fibrotic Lung Disease - Non-Specific Interstitial Pneumonia (NSIP)

Question 1

What is pneumonia?

1 - always an infection in the lung parenchymal tissue, leading to inflammation
2 - inflammation of the parenchymal tissue of the lungs
3 - inflammation of the extra pulmonary lung tissue
4 - all of the above

Answer 1

2 - inflammation of the parenchymal tissue of the lungs

• most common cause is an infection

Question 2

In a restrictive lung disease, do patients have difficulty in expiration, exhalation or both?

Answer 2

• inhalation

Question 3

In restrictive lung diseases the elastic tissue of the lungs is affected. Are both recoil and compliance of lung tissue reduced in asthma?

Answer 3

• no
• lost of elastic properties, so lungs cannot expand properly

Question 4

Looking at the alveolar in the image, label the image using the labels below:

• interstitial space
• type 1 pneumocytes
• type 2 pneumocytes
• pulmonary capillaries
• surfactant
• macrophages
• fibroblasts

Answer 4

1 - type 1 pneumocytes
2 - pulmonary capillaries
3 - macrophages
4 - fibroblasts
5 - interstitial space
6 - surfactant
7 - type 2 pneumocytes

Question 5

In spirometry, would we expect to see an increase of decrease in functional residual capacity (FRC) (remaining air in lungs at end of normal exhalation) in a patient with a restrictive lung disease?

Answer 5

• decreased as lungs are typically smaller
• recoil = increased (ability of lungs to snap back and exhale air)
• compliance = decreased (lungs unable to stretch)

Question 6

In spirometry, would we expect to see an increase of decrease in forced vital capacity (FVC) (air that can forcefully expired following maximum inhalation) in a patient with a restrictive lung disease?

Answer 6

• significant reduction
• compliance is reduced so patients lungs do not expand properly

Question 7

In spirometry, would we expect to see an increase of decrease in forced expiratory volume in 1 second (FEV1) (air that can forcefully expired in 1 second following maximum inhalation) in a patient with a restrictive lung disease?

Answer 7

• mildly reduced

Question 8

What is the ratio that is diagnostic in patients with a restrictive lung disease?

1 - FVC/FEV1 >90%
2 - FVC/FEV1 >80%
3 - FVC/FEV1 >75%
4 - FVC/FEV1 >60%

Answer 8

3 - FVC/FEV1 >75%
- both FVC and FEV1 are reduced but FVC is reduced more

Question 9

In patients with a restrictive lung disease is the total lung capacity increased or decreased?

Answer 9

• decreased
• lungs can become smaller

Question 10

There are 2 types of restrictive lung disease. Which 2 of the following are the correct categories of restrictive lung diseases?

1 - interstitial
2 - alveolar fibrosis
3 - extra pulmonary
4 - intra pulmonary

Answer 10

1 - interstitial
- lung tissue is affected and loses elastic properties

3 - extra pulmonary
- affects tissue outside the lungs, such as obesity and scoliosis

Question 11

What is non-specific interstitial pneumonia?

1 - fibrosis of lung tissue always caused by infection
2 - fibrosis of lung tissue always caused by occupational hazards
3 - form of idiopathic interstitial pneumonias
4 - all of the above

Answer 11

3 - form of idiopathic interstitial pneumonias

• has been linked with autoimmune disease

Question 12

What is the median age of patients with non-specific interstitial pneumonia?

1 - 20-30 y/o
2 - 30-40 y/o
3 - 40-50 y/o
4 - >65 y/o

Answer 12

3 - 40-50 y/o
- not linked to smoking

Question 13

Which of the following symptoms does NOT present in patients with non-specific interstitial pneumonia?

1 - progressive dyspnea over 1 years
2 - dry cough
3 - crackles
4 - weight gain
5 - fatigue

Answer 13

4 - weight gain
- patients typically lose weight, generally due to metabolic demand required to breathe

Question 14

All of the following conditions are associated with non-specific interstitial pneumonia, EXCEPT which one?

1 - scleroderma
2 - osteoarthritis
3 - rheumatoid arthritis
4 - systemic lupus erythematosus (SLE)
5 - collagen vascular disease

Answer 14

2 - osteoarthritis

• all other are connective tissue disorders

Question 15

Does non-specific interstitial pneumonia or idiopathic pulmonary fibrosis have a better prognosis?

Answer 15

• non-specific interstitial pneumonia
• better response to steroids and anti-inflammatory drugs

Question 16

Is non-specific interstitial pneumonia more common in men or women?

Answer 16

• both are equally affected

Question 17

Is non-specific interstitial pneumonia would we see enlarged or smaller lungs on a chest X-ray?

Answer 17

• reduced as it is a restrictive lung disease

Question 18

In a patient with non-specific interstitial pneumonia would we expect to see reticular nodules at base of lungs on a chest X-ray?

Answer 18

yes

Question 19

On a CT scan what would we expect to see in a patient with non-specific interstitial pneumonia?

1 - ground glass changes
2 - parenchymal changes
3 - hilar lymphadenopathy
4 - honeycombing and traction bronchial dilation

Answer 19

1 - ground glass changes

Question 20

All of the following drugs have been shown to cause pulmonary fibrosis, EXCEPT which one?

1 - Amiodarone
2 - Bleomycin
3 - Methotrexate
4 - Nitrofurantoin
5 - Ramipril

Answer 20

5 - Ramipril

Question 21

All of the following are treatments for non-specific interstitial pneumonia, EXCEPT which one?

1 - treatment of underlying cause
2 - immunotherapy
3 - lung transplant
4 - long term O2 therapy
5 - palliative care

Answer 21

3 - lung transplant