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Pathoma LAH > Pulmonary: 9.5: Restrictive dz > Flashcards

Pulmonary: 9.5: Restrictive dz Flashcards

1
Q

What is pneumoconiosis?

A

interstitial fibrosis due to chronic exposure to small, fibrogenic particles

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2
Q

Why do sarcoid pts have hypercalcemia?

A

the granulomas have 1-alpha-hydroxylase activity, which can activate vitamin D

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3
Q

What other tissues, besides the lungs, are commonly affected by sarcoidosis?

A
  • uvea (Uveitis)
  • skin (cutaneous nodules or erythema nodosum)
  • salivary and lacrimal glands
  • any other tissue
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4
Q

What is hypersensitivity pneumonitis?

A

a granulomatous reaction to inhaled organic antigens, esp from birds

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5
Q

Where do sarcoid granulomas occur in the lung?

A

in the hilar lymph nodes and throughout the lung tissue

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6
Q

What cells are assoc. with a granulomatous reaction?

A

eosinophils

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7
Q

What happens in idiopathic pulmonary fibrosis?

A

fibrosis of the lung interstitium

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8
Q

Pts exposed to asbestos are much more likely to get ____ than _____.

A

lung CA; mesothelioma

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9
Q

This is a systemic disease characterized by noncaseating granulomas in multiple organs.

A

sarcoidosis

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10
Q

What are the s/s of hypersensitivity pneumonitis?

A
  1. hours after exposure: fever, cough, dyspnea
  2. chronic: interstitial fibrosis
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11
Q

What workers are at risk of silicosis pneumoconiosis?

A

sandblasters and silica miners

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12
Q

Granulomas in the lung cause _____.

A

restrictive lung dz

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13
Q

What does non-caseating mean?

A

all the cells are alive; no necrosis

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14
Q

In sarcoid patients, granulomas have 1-alpha-hydroxylase activity, which can activate vitamin D. What does this cause?

A

hypercalcemia

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15
Q

What doe TGF-beta do?

A

induces a healing response and fibrosis

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16
Q

What are the clinical features of sarcoidosis?

A

cough, SOB, elevated serum ACE, hypercalcemia

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17
Q

What lung function values will be indicative of restrictive lung disease?

A
  • decreased TLC
  • decreased FVC
  • decreased FEV1
  • increased FEV1:FVC ratio (>80%)
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18
Q

This is a giant cell in sarcoidosis with an internal star-like pattern.

A

an asteroid body

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19
Q

Restrictive lung diseases cause _____ compliance.

A

decreased

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20
Q

What workers are at increased risk of asbestosis pneumoconiosis?

A

construction workers, plumbers, and shipyard workers

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21
Q

This is a granulomatous reaction to inhaled organic antigens, esp from birds.

A

hypersensitivity pneumonitis

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22
Q

Asbestosis pneumoconiosis pts are at increased risk of?

A

lung carcinoma and mesothelioma

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23
Q

What is an asbestos body?

A

a long, brown rod with iron depositions found in lung samples

24
Q

What are the clinical features of idiopathic pulmonary fibrosis?

A
  1. progressive dyspnea and cough
  2. fibrosis on lung CT beginning at the subpleura
25
Q

Pts with silicosis pneumoconiosis are at increased risk of _____.

A

TB

26
Q

What disease can sarcoidosis mimic?

A

Sjogren syndrome

27
Q

Where is TB found in the lung?

A

in the upper lobes

28
Q

What cytokine induces a healing response and fibrosis?

A

TGF-beta

29
Q

What are the pathologic findings of berylliosis pneumoconiosis?

A
  • noncaseating granulomas in the lung
  • hilar lymph nodes
  • systemic organs
30
Q
  1. DX?
  2. progressive dyspnea and cough
  3. fibrosis on lung CT beginning at the subpleura
A

idiopathic pulmonary fibrosis

31
Q

This is interstitial fibrosis due to chronic exposure to small, fibrogenic particles.

A

pneumoconiosis

32
Q

What is the tx for sarcoidosis?

A
  1. steroids
  2. none- self limiting and resolves spontaneously
33
Q

Fibrosis in pneumoconiosis is mediated by _____.

A

macs

34
Q

This is a long, brown rod with iron depositions found in certain lung histology.

A

asbestos bodies

35
Q

What is the cause of idiopathic pulmonary fibrosis?

A

unknown (that’s why it’s idiopathic)

36
Q

What is anthracosis?

A

carbon in the lung (black spots)- a little is normal

37
Q

Silica in the macs _____.

A

impair the phagolysosome formation

38
Q

What are the occupations most at risk for berylliosis pneumoconiosis?

A

miners, aerospace workers

39
Q

What disease is often associated with coal workers’ pneumoconiosis?

A

RA

40
Q

What are the pathologic findings of silicosis pneumoconiosis?

A

fibrotic nodules in the upper lobes

41
Q

What is an asteroid body?

A

a giant cell in sarcoidosis with an internal star-like pattern

42
Q

What are the interstitial diseases of the lung?

A
  1. idiopathic pulmonary fibrosis
  2. pneumoconiosis
  3. sarcoidosis
  4. hypersensitivity pneumonitis
43
Q

Pts with berylliosis pneumoconiosis are at increased risk for?

A

lung CA

44
Q

What causes pneumoconiosis?

A

occupational exposure

45
Q

Name 2 drugs that can induce interstitial fibrosis.

A
  1. bleomycin
  2. amiodarone
46
Q

What is sarcoidosis?

A

a systemic disease characterized by noncaseating granulomas in multiple organs

47
Q

Which pneumoconiosis is similar to sarcoidosis?

A

Berylliosis

48
Q

What is the defining cell of a granuloma?

A

an epitheliod hystiocyte

49
Q

What pneumoconiosis increases your risk of TB?

A

pneumoconiosis

50
Q

What is the interstitium?

A

the thin, delicate alveolar wall

51
Q

What are the pathologic findings in asbestosis pneumoconiosis?

A

fibrosis of the lung and pleura in plaques- may have long, golden brown fibers containing iron, which are asbestos bodies (diagnostic)

52
Q

What is the most common cause of restrictive lung disease?

A

interstitial fibrosis

53
Q

What is the treatment for idiopathic pulmonary fibrosis?

A

lung transplant

54
Q

What are the pathologic findins in Coal Workers’ pneumoconiosis?

A

diffuse anthracosis and fibrosis

55
Q

Name 4 exposures that can cause pneumoconiosis.

A
  1. coal/carbon
  2. silica
  3. beryllium
  4. asbestos

Pathoma LAH (77 decks)

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