Pulm Path Lecture 2 Flashcards

1
Q

What is the radiology finding associated with idiopathic pulmonary fibrosis?

A

Basilar infiltrates with progression to honeycomb lung.

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2
Q

What are the clinical findings associated with IPF?

A

Dyspnea, crackles, restrictive pattern on PFT.

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3
Q

What are the 3 contributing factors to IPF?

A
  1. ) Environmental
  2. ) Genetic
  3. ) Age >50
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4
Q

Diagnosis of IPF requires?

A

classic findings on high resolution CT or biopsy

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5
Q

Pulmonary biopsy of IPF shows?

A

Findings of usual interstitial pneumonia (UIP)

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6
Q

What are potential therapies for IPF?

A

Transplantation & drugs that block fibrosis

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7
Q

What makes non-specific interstitial pneumonia different than IPF?

A

It has a uniform pattern of fibrosis and inflammation. It also has a better prognosis

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8
Q

What is COP?

A

Cryptogenic Organizing Pneumonia

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9
Q

What is the histological feature of COP?

A

Masson bodies (organizing plugs of connective tissue)

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10
Q

How do you diagnose COP?

A

It is a diagnosis of exclusion

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11
Q

How do you treat COP?

A

Oral steroids. There is a good prognosis

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12
Q

What is the definition of sarcoidosis?

A

Systemic disease manifesting as non-caseating granulomas

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13
Q

Desquamative interstitial pneumonia shows?

A

stuffed alveolar spaces full of macrophages

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14
Q

What is the treatment for DSIP?

A

Stop smoking and corticosteroids

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15
Q

When does DSIP present?

A

In smokers in the 4th-5th decade

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16
Q

What is RB-ILD?

A

respiratory bronchiolitis - interstitial lung disease

17
Q

When does RB-ILD present?

A

earlier than DSIP, 3rd-4th decade of life

18
Q

Describe the 3 stages of RB-ILD

A
  1. ) fewer macrophages than DSIP
  2. ) peri bronchiolar metaplasia with abnormally located cilia cells
  3. ) Fibrosis in advanced cases
19
Q

Is RB-ILD reversible?

A

Yes, if caught early with smoking cessation

20
Q

LCH stands for what?

A

Langerhans Cell Histiocytosis

21
Q

What population is LCH present in?

A

Young smokers

22
Q

What does LCH look like histologically?

A

A stellate lung lesion with eosinophils, langerhans cells, and varying fibrosis and cysts

23
Q

What can patients with LCH present with?

A

pneumothorax

24
Q

What can LCH reverse with?

A

Smoking cessation

25
Q

LCH is what positive?

A

CD1a

26
Q

What is pulmonary alveolar proteinosis?

A

Impairment of surfactant metabolism due to defect of GM-CSF

27
Q

What happens in pulmonary alveolar proteinosis?

A

Accumulation of surfactant proteins throughout alveoli and airspaces