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Respiratory system 2017 > Pathology of restrictive lung disease > Flashcards

Pathology of restrictive lung disease Flashcards

1
Q

What is the lung interstitium?

A

Pulmonary interstitium is a collection of support tissues within the lung that includes the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues, which acts to form an airtight barrier

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2
Q

What happens in interstitial lung disease?

A

Normal alveolar walls become widened and thickened due to a pathological process leading to the formation of stiff, non-compliant lungs

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3
Q

What is the majority of the interstitium made up of?

A

Elastin - what is affected in interstitial lung disease

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4
Q

How does restrictive lung disease affect the spirometry values of the patient?

A

Both FEV1 and FVC are decreased, but the ratio is maintained at “healthy” 0.8 (80%)

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5
Q

What causes the reduced gas transfer in restrictive lung disease?

A

Seperation of capillary and alveolar epithelium

Increased diffusion distances in fibrosis

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6
Q

What is associated with diffuse alveolar damage?

A 
Major trauma
Chemical injury/toxic inhalation
Circulatory shock
Drugs
Infection
AI disease
Radiation
ARDS
Can be idiopathic
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7
Q

Describe the key characteristics of diffuse alveolar damage

A

Marked changes in blood pressure
Hyaline membrane production around alveoli
Accumulation of exudate in alveoli
Pulmonary oedema

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8
Q

Describe the histological features of diffuse alveolar damage

A 
Protein rich oedema
Fibrin
Hyaline membranes around alveoli
Denuded basement membrane 
Epithelial + fibroblast proliferation
Fibrous scarring of tissue
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9
Q

Describe sarcoidosis

A

Multisystem granulomatous disease causing epitheliod and multinucleate giant cell granulomas, where necrosis or caseation is unusual

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10
Q

What group is most associated with sarcoidosis?

A

Young females, northern europeans or afro-caribeans

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11
Q

What organs are usually affected in sarcoidosis?

A 
Lungs
Spleen
Liver
Skin
Eyes
Skeletal muscle
Bone marrow
Salivary glands
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12
Q

How do those with sarcoidosis usually present?

A 
Painful joints
Erythema nodosum (tender red bumps)
Bilateral hilar lymphadenopathy
Abnormal CXR
SOB
Cough
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13
Q

Describe some blood results typical of sarcoidosis

A

Increased calcium
Normal PTH
Increased ACE

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14
Q

What is the Kveim test?

A

Test for sarcoidosis - inject cells from liver of sarcoidosis patient into skin, if they develop a granuloma at the site they are positive for sarcoidosis

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15
Q

How can we rule out TB in sarcoidosis?

A

If tissue is necrotic
Tuberculin/mantoux test
Kveim test

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16
Q

What is hypersensitivity pneumonitis?

A

Inflammation of the alveoli caused by type III or IV hypersensitivity reactions to inhaled organic dusts

17
Q

Where is the main site of reaction in hypersensitivity pneumonitis?

A

Hilar regions, where the inhaled agents are usually deposited

18
Q

What are some common antigens which cause hypersensitivity pneumonitis?

A 
Thermophilic actinomycetes (mould hay)
Micropolyspora faeni
Thermactinomyces vulgaris
Bird/animal proteins
Fungi
Chemicals
19
Q

Describe the symptoms of acute hypersensitivity pneumonitis

A 
Fever
Dry cough
Myalgia
Crackles on inspiration
Tachypnoea
Wheeze
Precipitating antibody
20
Q

Describe the symptoms of chronic hypersensitivity pneumonitis

A 
Malaise
SOB
Cough
Low grade illness
Crackles on inspiration
Some wheeze
Can lead to respiratory failure
Cyanosis
Finger clubbing
21
Q

Describe the histopathology of hypersensitivity pneumonitis

A 
Evidence of diffuse chronic inflammation
Soft centriacinar granulomata
Bilateral
Interstitial pneumonitis
Alveolar septal thickening (infiltrates)
Foamy histiocytes
22
Q

Describe usual interstitial pneumonitis

A

Patchy interstitial chronic inflammation, resulting in type II pneumocyte hyperplasia and smooth muscle and vascular proliferation due to some sort of injury. Results in honeycomb lung and cobblestoning

23
Q

What can cause usual interstitial pneumonitis?

A

Connective tissue diseases
Drug reactions
Post infection
Industrial exposure

Most cases are cryptogenic or idiopathic and are therefore called either:

  • cryogenic fibrosing alveolitis
  • idiopathic pulmonary fibrosis
24
Q

Describe the typical usual interstitial pneumonitis patient

A

Elderly male

Poor prognosis

25
Q

What are the symptoms of usual interstitial pneumonitis?

A 
Dyspnoea
Cough
Basal crackle
Cyanosis
Clubbing
26
Q

What can be seen on CXR of usual interstitial pneumonitis?

A

Basal/posterior disease
Diffuse infiltrates
Cysts
Ground glass opacities

Respiratory system 2017 (31 decks)

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