B4-056 Restrictive Pulmonary Disease Flashcards

1
Q
  • disorders involving inflammation and fibrosis of the lung parenchyma, particularly the interstitium
  • causes reduced diffusion capacity
A

interstitial lung disease

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2
Q

space between the epithelial and endothelial basement membranes in the alveolar wall

A

interstitium

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3
Q

fibrosing interstitial lung diseases

just the 3 she said we’re responsible for

A
  • idiopathic pulmonary fibrosis
  • non-specific interstitial pneumonia
  • pneumoconiosis
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4
Q

insidious onset of dyspnea on exertion
dry cough
hypoxemia, cyanosis, clubbing
inspiratory crackles

A

idiopathic pulmonary fibrosis

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5
Q

CT findings

  • bilateral basilar-predominant reticulation and bronchiectasis
  • bilateral subpleural honeycomb cysts
A

idiopathic pulmonary fibrosis

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6
Q

chronic fibrosing interstitial lung disease with high mortality

A

idiopathic pulmonary fibrosis

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7
Q
  • female
  • non-smoker
  • dyspnea, cough of several months duration
A

non specific interstitial pneumonia

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8
Q
  • interstitial fibrosis and inflammation
  • lung architecture preserved
  • no honeycomb/fibroblast foci
A

non specific interstitial pneumonia

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9
Q

temporal homogenity

A

non specific interstitial pneumonia

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10
Q
  1. Patchwork of interstitial fibrosis
  2. septal distribution
  3. dense fibrosis/honeycombing
  4. fibroblast foci
A

UIP

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11
Q

inflammatory reaction of the lung to inhaled dust/mineral particles

A

pneumoconiosis

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12
Q

proinflammatory cystalling hydrated silicates

A

asbestos

pneumoconiosis

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13
Q

golden brown beaded rods with translucent center

A

ferruginous body

asbestos pneumoconiosis

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14
Q

caused by inhalation of proinflammatory crystalline sillicon dioxide

A

silicosis

pneumoconiosis

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15
Q

most prevalent occupation disease worldwide

concrete, stone cutting, sand blasting, hard rock mining

A

silicosis

pneumoconiosis

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16
Q

pneumoconiosis causes activation of

A

inflammasome

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17
Q

bright crystals under polarized light

A

silicosis

pneumoconiosis

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18
Q
  • alveolar proteinosis
  • cholesterol clefts
  • eosinophilic globules
A

acute silicosis

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19
Q

inhaled carbon dust engulfed by alveolar or interstitial macrophages

A

anthracosis

coal workers pneumoconiosis

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20
Q

coal macules and nodules consisting of carbon laden macrophages and scar

A

simple coal workers pneumoconiosis

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21
Q
  • large scars with dense collagen and black pigment
  • progressive massive fibrosis
A

complicated coal workers pneumoconiosis

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22
Q

granulomatious interstitial lung diseases

2

A
  • sarcoidosis
  • hypersensitivity pneumonitis
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23
Q
A

sarcoidosis

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24
Q
  • multi-nucleated giant cells
  • epithelioid
  • non-necrotizing
  • hyalinizing fibrosis
A

sarcoidosis

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25
Q
  • high ACE level
  • hypercalcemia
  • hypercalciuria
A

sarcoidosis

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26
Q

from immune reactiong provoked by inhaled antigen in susceptible individuals

A

hypersensitivity pneumonitis

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27
Q
  • increased CD4 and CD8 T lymphocytes
  • non-necrotizing granulomas
  • antigen specific IgG antibodies
  • complement and Ig deposits
A

hypersensitivity pneumonitis

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28
Q
  • insidious onset SOB, cough, weight loss, exercise intolerance
  • acute episodes following inhalation of irritant w/ fever, cough, elevated WBC
A

hypersensitivity pneumonitis

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29
Q
  • mosaic perfusion
  • BAL fluid >20% lymphocytes
A

hypersensitivity pneumonitis

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30
Q

interstitial poorly formed non-necrotizing granulomas

A

hypersensitivity pneumonitis

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31
Q

characterized by proliferation of Langerhans cells

A

pulmonary langerhans cell histiocytosis

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32
Q
  • smoking related
  • BRAF or MPK connection
A

pulmonary langerhans cell histiocytosis

33
Q
  • peribronchial nodules on CT, upper lobe predominate
  • bird beak or tennis racket on electron microscopy
A

pulmonary langerhans cell histiocytosis

34
Q

eosinophilic granuloma

A

pulmonary langerhans cell histiocytosis

35
Q

CD1a, S100, Langerin positive

A

pulmonary langerhans cell histiocytosis

36
Q

generally improves after smoking cessation, barring BRAF/MPK mutation

A

pulmonary langerhans cell histiocytosis

37
Q

airspace filling lung diseases

2

A
  • pulmonary alveolar proteinosis
  • cryptogenic organizing pneumonia
38
Q
  • positive periodic acid stain
  • accumulation of surfactant in airspaces
A

pulmonary alveolar proteinosis

39
Q
  • dyspnea
  • productive cough
  • gelatinous sputum
A

pulmonary alveolar proteinosis

40
Q

CT finding

crazy paving

A

pulmonary alveolar proteinosis

41
Q

whorled lamellar bodies in type 2 pneumocytes on electron microscopy

A

pulmonary alveolar proteinosis

42
Q
  • polypoid branching plugs (Masson bodies)
  • mild inflammation
  • preserved architecture
A

organizing pneumonia

43
Q
  • subacute cough with dyspnea
  • patchy bilateral ground glass opacities
  • idopathic
A

cryptogenic organizing pneumonia

44
Q

destruction of bronchial walls by recurrent inflammation

A

bronchiectasis

45
Q

irreversible dilation of bronchi from inflammation and destruction of bronchial walls following prolonged infections/obstructions

A

bronchiectasis

46
Q

unopposed action of elastases damaging alveolar tissue

A

emphysema

47
Q

bronchial mucosa damage associated with eosinophils

A

bronchial asthma

48
Q

destruction of the walls of distal airspaces without prominent fibrosis

A

emphysema

49
Q

associated with nasal polyps, chronic rhinitis, severe asthma and intolerance to aspirin

A

drug induced asthma

50
Q

chronic productive cough without cause for > 3months in a 2 year period

A

chronic bronchitis

51
Q

90% of all cases occur in smokers

A

chronic bronchitis

52
Q
  • hypertrophy of bronchial submucosal glands and hyperplasia of goblet cells
  • excessive mucus production and airway obstruction
A

chronic bronchitis

53
Q

thickening of the bronchial epithelial basement membrane and hypertrophy of bronchial smooth muscle

A

atopic asthma

54
Q

contact with allergen results in degranulation of mast cells and release of histamines

A

atopic asthma

55
Q

dilation of respiratory bronchioles

A

centrilobar emphysema

56
Q

bronchial dilation with inflammatory destruction

A

bronchiectasis

57
Q

most patient clinically diagnosed with emphysema younger than 40

A

A1AT deficiency

58
Q
  • charcot leyden crystals
  • curshmann spirals
  • inflammatory cells in mucus
A

atopic asthma

59
Q

marked thickening of pulmonary arteries

A

pulmonary hypertension

60
Q

will have characteristics of both obstructive and restrictive

A

pulmonary hypertension

61
Q

destruction of tissue in alveolar wall

A

emphysema

62
Q

fibrosis of alveolar wall

A

restrictive diseases

63
Q

non-necrotizing granulomas with multisystem involvement

A

sarcoidosis

64
Q

increased susceptibility to mycobacterium

A

silicosis

65
Q

most prevalent occupational disease worldwide

A

silicosis

66
Q

hypersensitivity pneumonitis caused by inhalation of actinomycete spores

A

farmer lung

pneumonitis

67
Q

neoplastic spindle and epitheliod cells that invade adipose tissue

A

asbestos

68
Q

polypoid plugs of organizing connective tissue

A

cryptogenic organizing pneumonia

69
Q

in-alveolar accumulation of granular eosinophilic material due to build up of surfactant

A

pulmonary alveolar proteinosis

70
Q

hyaline membranes lining alveolar space

A

diffuse alveolar damage

71
Q

abnormal dilation of distal airspaces

A

emphysema

72
Q

temporally heterogenous fibrosis with fibroblast foci and interstitial fibrosis

A

UIP

73
Q

firm, tan mass encasing the pleura

A

malignant meothelioma

74
Q

diaphragmatic calcified plaques

A

mesothelioma

75
Q

localized bronchial dilation due to chronic or recurrent infection

A

bronchiectasis

76
Q

immature dendritic cells with grooved, identical nuclei

A

langerhans

77
Q

positive stain for S100 and CD1a

A

langerhans

78
Q

after quitting smoking, patients with Langerhans cell histiocytosis

A

usually recover