2.11 - Pathology Of Restrictive Lung Disease

Question 1

What are the three types of bronchi?

Answer 1

Main (primary)
Lobar (secondary)
Segmental (tertiary)

Question 2

What are the three types of bronchiole?

Answer 2

Bronchiole
Terminal bronchiole
Respiratory bronchiole

Question 3

What three parts of the lung/thorax can be affected and lead to restrictive lung disease?

Answer 3

Disorders of the chest wall: neuromuscular disorders, severe obesity, kyphoscoliosis
Disorders of the pleura
Diseases of the lung: acute (diffuse alveolar damage), chronic (pulmonary fibrosis, granulomatous inflammation, eosinophillic etc.)

Question 4

Describe Acute Respiratory Distress Syndrome (ARDS)

Answer 4

Diffuse Alveolar Capillary Damage
Component of Shock, Multisystem organ failure
Severe life threatening respiratory insufficiency, cyanosis and arterial hypoxemia

Question 5

Describe the pathogenesis of ARDS

Answer 5

Capillary endothelial damage
Alveolar damage
Release of cytokines
Release of interleukins
Activation of neutrophils (release proteases, oxidants)
Increased vascular permeability
Exudation of fluid- alveolar flooding
Decreased Diffusion

Question 6

What are the outcomes of ARDS?

Answer 6

Respiratory acidosis
Death (60%)
Scarring - poorly aerated fibroses lung

Question 7

What are some signs and symptoms of chronic restrictive lung disease?

Answer 7

Clinical signs and symptoms
– Dyspnea (different onsets, +/- progressive) – Tachypnea
– Inspiratory crackles
– Reduced lung compliance and volume

Radiologic alteration 
– Distribution important 
– Irregular lines
– Small nodules
– Ground glass shadows

Question 8

Describe the characterisation of chronic restrictive lung disease

Answer 8

Characterized by diffuse and chronic involvement of pulmonary connective tissue (Interstitium)= interstitial lung diseases

Question 9

Describe the pathogenesis of CRLD

Answer 9

Similar Pathogenesis to ARDS
– Different mechanisms leading to inflammation of alveoli
– Accumulation of inflammatory cells in the alveolar walls and spaces
– Release of mediators (cytokines and interleukins)
– Alveolar wall damage
– Fibrosis of the alveolar walls (IRREVERSIBLE/POOR PROGNOSIS)

Question 10

What are some complications of chronic restrictive lung disease?

Answer 10

End stage honey comb lung
Traction bronchiectasis
Pulmonary hypertension
Right sided heart failure (Cor pulmonale)

Question 11

What are some factors that affect the development of lung disease?

Answer 11

Duration and length of exposure
Amount of retained dust
Size (small 1-5 microns- can reach and settle in small alveoli)
Shape, buoyancy of the particles
Particle solubility (insoluble particles can remain in the lungs for years)
Additional irritants (SMOKING!!!!)
Preexisting lung disease

Question 12

What is a granuloma?

Answer 12

A focus of chronic inflammation

Comprised of activated macrophages surrounded by a collar of lymphocytes and plasma cells

Question 13

Why do granulomas form?

Answer 13

Granulomas form when the immune system fends off and isolates a poorly degradable or particulate antigen.