Restrictive Lung Diseases Flashcards

1
Q

What comprises the interstitium of the lung?

A

The connective tissue space around airways and vessels
AND
The space between the basement membranes of the alveolar walls

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2
Q

What are the general signs of restrictive lung diseases?

A
  • Reduced lung compliance
  • Low FEV1 and FVC (normal ratio)
  • Reduced Gast Transfer
  • V/Q mismatch
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3
Q

What is the typical presentation of diffuse lung disease?

A
  • Abnormal CXR
  • Dyspnoea
  • Type I Respiratory - Failure
  • Heart Failure
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4
Q

What does parenchymal lung injury lead to in an acute response?

A

Diffuse alveolar damage (DAD)

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5
Q

What non-idiopathic causes of diffuse alveolar damage are there?

A
  • Major trauma
  • Chemical injury
  • Circulatory shock
  • Drugs
  • Infection
  • Autoimmune disease
  • Radiation
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6
Q

What two phases of diffuse alveolar damage are there?

A

Exudative and proliferative

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7
Q

What are the histological features of DADS?

A
  • Protein rich oedema
  • Hyaline membranes
  • Denuded basement membranes
  • Epithelial and fibroblast proliferation
  • Fibrosis
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8
Q

What three responses to chronic parenchymal lung injury are there?

A
  • Usual Interstitial Pneumonitis (UIP)
  • Granulomatous responses
  • Other patterns
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9
Q

What granulomatous disorders occur?

A
  • Sarcoidosis

- Hypersensitivity pneumonitis

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10
Q

What is sarcoidosis?

A

A multisystem granulomatous disorder of unknown aetiology

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11
Q

What is the typical histopathology of sarcoidosis?

A
  • Epithelioid and giant cell granulomas
  • Necrosis and caseation (formation of hard tissue in tuberculosis) unusual
  • Little lymphoid infiltration
  • Variable associated fibrosis
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12
Q

What sex is most effected by sarcoidosis?

A

Females more than males

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13
Q

What are common organs that sarcoidosis infects?

A

Lymph nodes, lung, spleen, liver, eyes

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14
Q

What is the typical presentation of sarcoidosis in your adults?

A
  • Acute arthralgia (joint pain)
  • Erythema nodosum (red blotches in skin)
  • Bilateral hilar lymphadenopathy)
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15
Q

What treatment is used for sarcoidosis?

A

Corticosteroids

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16
Q

What antigens are responsible for hypersensitivity pneumonitis?

A
  • Thermophilic actinomycetes
  • Bird/animal proteins
  • Aspergillus spp.
  • Chemicals
17
Q

What is the other name for Hypersensitive Pneumonitis?

A

Extrinsic Allergic Alevolitis (EAA)

18
Q

What are the acute presentations of HP?

A

Fever, dry cough, myalgia (muscle pain), Chills, crackles, tachypnoea, wheeze

19
Q

What are the chronic presentations of HP?

A

Insidious, malaise, SOB, cough, Low grade illness, crackles and some wheeze

20
Q

What does cryptogenic mean? How does it differ from idiopathic?

A

Idiopathic - no cause

Whereas cryptogenic means there is a cause but it is unknown

21
Q

What is the histopathology associated with UIP?

A
  • Patchy interstitial chronic inflammation
  • Type II pneumocyte hyperplasia
  • Smooth muscles and vascular proliferation
  • Evidence of recent/old injury
22
Q

What is the common end state of each of the three results of chronic parenchymal lung tissue damage?

A

End stage lung fibrosis (honeycombing)