Week 5 - Pathology of Restrictive Lung Diseases Flashcards

1
Q

How does restrictive lung disease affect you?

A

decreased compliance (stretchability) in lung

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2
Q

How is gas transfer impaired? (3)

A
  • reduced gas transfer due to diffusion abnormality.
  • ventilation-perfusion mismatch.
  • high FVC/FEV1 ratios but low values due to less air to begin
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3
Q

What is the presentation of restrictive lung disease?

A
  • abnormal CXR/CT - lung length shortened.
  • Dyspnoea,
  • type 1 resp failure,
  • heart failure
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4
Q

What is DAD

A

individual has acute interstitial pneumonia. diffuse alveolar damage, also known as ARDS

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5
Q

What is the pathology of DAD?

A

protein rich fluid fills alveoli and causes inflammation and fibrosis, damaging it. Forms very solid lung.

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6
Q

What is the development of DAD associated with?

A
  • major trauma,
  • chemical injury toxic inhalation,
  • circulatory shock,
  • drugs,
  • infection,
  • autoimmune disease.
  • can be idiopathic
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7
Q

What is sarcoidosis?

A

idiopathic. type IV hypersensitivity. causes non-caesiating granulomas, nearly always in lymph, and very commonly in lungs

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8
Q

What is the presentation and treatment of sarcoidosis?

A

young adult with abnormal CXR or CT, SoB and cough, erythema nodosum and acute arthralgia.

corticosteroid treatment

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9
Q

What is Hypersensitivity Pneumonitis?

A

a response to antigens, typically farmers lung, bacteria, fungi, chemicals, bird/animal proteins. causes type III and IV hypersensitivity reaction - inflammation around granuloma

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10
Q

What is the symptomatic presentation of Hypersensitivity Pneumonitis?

A

dry cough, chills, fever, wheeze, crackles, tachypnoea, myalgia

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11
Q

Where are hypersensitivity pneumonitis granulomas seen and why?

A

apex of lung as higher O2 tension and later lymph node drainage.

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12
Q

What is Usual interstitial pneumonitis and when may you see this condition?

A

progressive scarring of the lungs.

may be seen in connective tissue diseases, drugs, asbestos, viruses

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13
Q

how do you differentiate between sarcoidosis and hypersensitivity pneumonitis?

A

both produce non-cassiating granulomas. sarcoidosis is type IV hypersensitivity reaction while hypersensitivity pneumonitis is type III and IV.

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14
Q

What is type III vs type IV hypersensitivity reaction?

A

Type III involves immune complex formation - granuloma. Type IV involves lymphocyte accumulation

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15
Q

What is the histopathology of usual interstitial pneumonitis? (UIP)

A

Patchy interstitial chronic inflammation. type II pneumocytes proliferate, and so do muscular and angiogenesis. fibroblasts activity proliferate - more areas become fibrous

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16
Q

What are symptoms of usual interstitial pneumonitis?

A

varies.
- mostly males >50.
- cough,
- dyspnoea,
- basal crackles,
- cyanosis,
- clubbing

17
Q

What is the clinical presentation of usual interstitial pneumonitis? (3)

A
  • CXR - basal and posterior fibrosis with honeycombing.
  • restrictive peak flow,
  • reduced gas transfer.
18
Q

What is the prognosis of usual interstitial pneumonitis?

A

progressive so poor - most dead within 5 years