Restrictive Lung Diseases (L19) Flashcards

1
Q

List the features of Restrictive Lung diseases.

6

A
  • Increased Lung Density
  • Lung is Stiff
  • Reduced Compliance
  • Breathlessness (Dyspnoea)
  • Greater Effort to Inflate Lungs
  • Abnormality of Alveolar Walls which renders them rigid
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2
Q

An increase in interstitial fluid or fibrosis produces what?

A

A Stiff lung.

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3
Q

What is ARDS?

What is it characterised by?
2

A
  • 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.
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4
Q

List the risk factors of ARDS.

6

A
  • 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
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5
Q

List the features of ARDS.

7

A
  • 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)
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6
Q

What is the ARDS mortality rate?

A

40%.

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7
Q

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

A
  • Lung tissue is damaged
  • Walls of the acini become inflamed
  • Fibrosis begins in the interstitium and the lungs become stiff
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8
Q

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

A

Interstitial Lung Disease.

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9
Q

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.
A

Chronic Restrictive Lung Diseases.

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10
Q

___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___.

A
  1. Interstitial
  2. Inflammatory
  3. Fibrosis
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11
Q

In terms of Interstitial Lung Diseases;

Inflammation of the bronchioles=?

A

Bronchiolitis.

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12
Q

In terms of Interstitial Lung Diseases;

Inflammation of the alveoli=?

A

Alveolitis.

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13
Q

In terms of Interstitial Lung Diseases;

Inflammation of the capillaries=?

A

Vasculitis.

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14
Q

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

Otherwise known as?

A

15.

Cryptogenic fibrosing alveolitis.

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15
Q

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

A
  • Usual Interstitial Pneumonitis (UIP)
  • Desquamative Interstitial Pneumonitis (DIP)
  • Acute Interstitial Pneumonitis (AIP)
  • Non-Specific Interstitial Pneumonitis
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16
Q

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

A

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).

17
Q

List the symptoms of Usual Interstitial Pneumonitis (UIP).

5

A
  • Persistent cough
  • Dyspnoea
  • Fatigue
  • Fever
  • Joint symptoms are common and finger clubbing occurs in 50% of cases.
18
Q

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

A

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

19
Q

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

A
  • Slow progressive dyspnea
  • Non-productive cough
  • Clubbing
20
Q

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

A

Hamman-Rich syndrome.

21
Q

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

A

Rapidly progressive interstitial pulmonary fibrosis.

22
Q

List the symptoms of Acute Interstitial Pneumonitis (AIP).

A
  • Rapidly progressive dyspnoea
  • Hypoxemia
  • Respiratory failure
23
Q

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

Describe.

A
  • 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
24
Q

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

A

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

25
Q

Describe Bronchiolitis Obliterans.

A

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

26
Q

Describe Sarcoidosis.

A

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