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
Pts with silicosis pneumoconiosis are at increased risk of \_\_\_\_\_.
TB
26
What disease can sarcoidosis mimic?
Sjogren syndrome
27
Where is TB found in the lung?
in the upper lobes
28
What cytokine induces a healing response and fibrosis?
TGF-beta
29
What are the pathologic findings of berylliosis pneumoconiosis?
* noncaseating granulomas in the lung * hilar lymph nodes * systemic organs
30
1. DX? 2. progressive dyspnea and cough 3. fibrosis on lung CT beginning at the subpleura
idiopathic pulmonary fibrosis
31
This is interstitial fibrosis due to chronic exposure to small, fibrogenic particles.
pneumoconiosis
32
What is the tx for sarcoidosis?
1. steroids 2. none- self limiting and resolves spontaneously
33
Fibrosis in pneumoconiosis is mediated by \_\_\_\_\_.
macs
34
This is a long, brown rod with iron depositions found in certain lung histology.
asbestos bodies
35
What is the cause of idiopathic pulmonary fibrosis?
unknown (that's why it's idiopathic)
36
What is anthracosis?
carbon in the lung (black spots)- a little is normal
37
Silica in the macs \_\_\_\_\_.
impair the phagolysosome formation
38
What are the occupations most at risk for berylliosis pneumoconiosis?
miners, aerospace workers
39
What disease is often associated with coal workers' pneumoconiosis?
RA
40
What are the pathologic findings of silicosis pneumoconiosis?
fibrotic nodules in the upper lobes
41
What is an asteroid body?
a giant cell in sarcoidosis with an internal star-like pattern
42
What are the interstitial diseases of the lung?
1. idiopathic pulmonary fibrosis 2. pneumoconiosis 3. sarcoidosis 4. hypersensitivity pneumonitis
43
Pts with berylliosis pneumoconiosis are at increased risk for?
lung CA
44
What causes pneumoconiosis?
occupational exposure
45
Name 2 drugs that can induce interstitial fibrosis.
1. bleomycin 2. amiodarone
46
What is sarcoidosis?
a systemic disease characterized by noncaseating granulomas in multiple organs
47
Which pneumoconiosis is similar to sarcoidosis?
Berylliosis
48
What is the defining cell of a granuloma?
an epitheliod hystiocyte
49
What pneumoconiosis increases your risk of TB?
pneumoconiosis
50
What is the interstitium?
the thin, delicate alveolar wall
51
What are the pathologic findings in asbestosis pneumoconiosis?
fibrosis of the lung and pleura in plaques- may have long, golden brown fibers containing iron, which are asbestos bodies (diagnostic)
52
What is the most common cause of restrictive lung disease?
interstitial fibrosis
53
What is the treatment for idiopathic pulmonary fibrosis?
lung transplant
54
What are the pathologic findins in Coal Workers' pneumoconiosis?
diffuse anthracosis and fibrosis
55
Name 4 exposures that can cause pneumoconiosis.
1. coal/carbon 2. silica 3. beryllium 4. asbestos