Restrictive Lung Diseases (L19)

Question 1

List the features of Restrictive Lung diseases.

6

Answer 1

• Increased Lung Density
• Lung is Stiff
• Reduced Compliance
• Breathlessness (Dyspnoea)
• Greater Effort to Inflate Lungs
• Abnormality of Alveolar Walls which renders them rigid

Question 2

An increase in interstitial fluid or fibrosis produces what?

Answer 2

A Stiff lung.

Question 3

What is ARDS?

What is it characterised by?
2

Answer 3

• ARDS is a syndrome of acute and persistent inflammatory disease of the lungs and is characterised by:
• Bilateral inflammatory infiltrates
• Ratio of partial pressure of arterial oxygen to the fractional concentration of inspired air (Pa02/FiO2) of less than 200 mmHg.

Question 4

List the risk factors of ARDS.

6

Answer 4

• Sepsis (the most common cause)
• Massive trauma with shock and multiple transfusions
• Hypovolaemic shock (the volume of circulatory sytstem is depleted thus there is limited circulation to the tissues)
• Pneumonia
• Smoke Inhalation
• Drugs – paraquat, heroin, asprin

Question 5

List the features of ARDS.

7

Answer 5

• Diffuse alveolar damage
• Hyaline membranes line the respiratory
  bronchioles
• Severe injury to alveolar capillary walls
• Tachypnea (abnormally fast breathing) and Dyspnoea.
• Pulmonary Oedema
• Arterial Hypoxaemia
• Alveolar flooding and Atelectasis (due to loss of surfactant)

Question 6

What is the ARDS mortality rate?

Answer 6

40%.

Question 7

When a person has Interstitial Lung disease (ILD) the lung is affected in what three ways?

Answer 7

• Lung tissue is damaged
• Walls of the acini become inflamed
• Fibrosis begins in the interstitium and the lungs become stiff

Question 8

_____ _____ _____ is a common term for more than 130 chronic lung diseases.

Answer 8

Interstitial Lung Disease.

Question 9

Name the disease grouping.

• These develop over months/years and leads to a slowly decreasing respiratory efficiency.
• With chronic interstitial fibrosis leads to a ‘honeycomb’ lung.
• There is an infiltration of macrophages and microcyst formation.
• Clinically patients exhibit dyspnoea, cough and in advanced cases hypoxemia and cyanosis.

Answer 9

Chronic Restrictive Lung Diseases.

Question 10

___1___ lung disease has traditionally been thought to occur as a result of an initial injury to the lung that causes the recruitment of ___2___ cells, release of cytokines and eventually, from increased fibroblast activity parenchymal remodelling and ___3___.

Answer 10

1. Interstitial
2. Inflammatory
3. Fibrosis

Question 11

In terms of Interstitial Lung Diseases;

Inflammation of the bronchioles=?

Answer 11

Bronchiolitis.

Question 12

In terms of Interstitial Lung Diseases;

Inflammation of the alveoli=?

Answer 12

Alveolitis.

Question 13

In terms of Interstitial Lung Diseases;

Inflammation of the capillaries=?

Answer 13

Vasculitis.

Question 14

Idiopathic pulmonary fibrosis (IPF) affects about __/100 000 people.

Otherwise known as?

Answer 14

15.

Cryptogenic fibrosing alveolitis.

Question 15

List the 4 types of Idiopathic pulmonary fibrosis (IPF).

Answer 15

• Usual Interstitial Pneumonitis (UIP)
• Desquamative Interstitial Pneumonitis (DIP)
• Acute Interstitial Pneumonitis (AIP)
• Non-Specific Interstitial Pneumonitis

Question 16

Describe the most outstanding feature of Usual Interstitial Pneumonitis (UIP).

Answer 16

Temporal heterogeneity meaning a biopsy obtained on a given day may show two or more different developmental features plus a non pathological feature (normal lung interspersed with diseased areas).

Question 17

List the symptoms of Usual Interstitial Pneumonitis (UIP).

5

Answer 17

• Persistent cough
• Dyspnoea
• Fatigue
• Fever
• Joint symptoms are common and finger clubbing occurs in 50% of cases.

Question 18

Describe the most outstanding feature of Desquamative Interstitial Pneumonitis (DIP).

Answer 18

The presence of large numbers of macrophages in the alveolar spaces, some with a brown cytoplasmic pigmentation.

Question 19

List the symptoms of Desquamative Interstitial Pneumonitis (DIP).
(3)

Answer 19

• Slow progressive dyspnea
• Non-productive cough
• Clubbing

Question 20

What is the other name for Acute Interstitial Pneumonitis (AIP)?

Answer 20

Hamman-Rich syndrome.

Question 21

Describe the most outstanding feature of Acute Interstitial Pneumonitis (AIP).

Answer 21

Rapidly progressive interstitial pulmonary fibrosis.

Question 22

List the symptoms of Acute Interstitial Pneumonitis (AIP).

Answer 22

• Rapidly progressive dyspnoea
• Hypoxemia
• Respiratory failure

Question 23

Histologically, what is the hallmark of Acute Interstitial Pneumonitis (AIP)?

Describe.

Answer 23

• Diffuse alveolar damage (DAD)
• DAD manifests initially as injury to the alveolar lining and endothelial cells, pulmonary oedema and hyaline membrane formation. On X-ray there is patchy airspace opacifications

Question 24

Describe the most outstanding feature of Non-Specific Interstitial Pneumonitis.

Answer 24

Diffuse interstitial thickening with inflammatory cells and fairly good preservation of the alveolar architecture.

Question 25

Describe Bronchiolitis Obliterans.

Answer 25

Characterised by formation of fibrous tissue within the lumina of small bronchioles and alveoli. Very sensitive to corticosteroid therapy.

Question 26

Describe Sarcoidosis.

Answer 26

A relatively common granulatomatas disease. In many cases the disease is asymptomatic although lung destruction and fibrosis may occur.