Ch. 27 Flashcards

1
Q

What are other names of interstitial lung disease?

A

diffuse interstitial lung disease, fibrotic interstitial lung disease, and pul- monary fibrosis)

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2
Q

More than how many disease entities are characterized by acute, subacute, or chronic inflammatory infiltration of alveo- lar walls by cells, fluid, and connective tissue.

A

180

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3
Q

If untreated the inflammatory process can progress to what?

A

Pulmonary fibrosis

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4
Q

What do the anatomic alterations of the lungs involve?

A

the bronchi, alveolar walls, and adjacent alveolar spaces.

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5
Q

In severe cases what can the extensive inflammation lead too?

A

pulmonary fibrosis, granulomas, honeycombing, and cavitation.

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6
Q

What are the major pathologic or structural changes associated with chronic ILDs

A

•Destruction of the alveoli and adjacent pulmonary
capillaries
• Fibrotic thickening of the respiratory bronchioles, alveolar ducts, and alveoli
• Granulomas
• Honeycombing and cavity formation
• Fibrocalcific pleural plaques (particularly in asbestosis)
• Bronchospasm
• Excessive bronchial secretions (caused by inflammation of
airways)

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7
Q

What is asbestos?

A

Exposure to asbestos may cause asbestosis—a common form of ILD. Asbestos fibers are a mixture of fibrous minerals composed of hydrous silicates of magnesium, sodium, and iron in various proportions.

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8
Q

What are the 2 primary types?

A

Amphiboles and chrysotile

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9
Q

How long are asbestos fibers?

A

50 to 100 μm in length and are about 0.5 μm in diameter.

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10
Q

Which lobes are mostly affected?

A

Lower lobes

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11
Q

T or F; Pleural calcification is common and diagnostic in patients with an asbestos exposure history.

A

True

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12
Q

What is coal worker pneumonia?

A

The pulmonary deposition and accumulation of large amounts of coal dust

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13
Q

What is CWP also know as?

A

coal miner’s lung and black lung

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14
Q

How is CWP characterized?

A

by the presence of pinpoint nodules called coal macules (black spots)

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15
Q

What is focal emphysema?

A

The coal macules often develop around the first- and second-generation respiratory bronchioles and cause the adjacent alveoli to retract.

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16
Q

What is Complicated CWP or progressive massive fibrosis (PMF)?

A

areas of fibrotic nodules greater than 1 cm in diameter.

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17
Q

What are the fibrotic changes in CWP usually caused by?

A

Silica

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18
Q

What are other names for silicosis?

A

grinder’s disease or quartz silicosis

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19
Q

What is silicosis caused by?

A

chronic inhalation of crystalline, free silica, or silicon dioxide particles.

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20
Q

Silica is the main component of more than how many of the rocks on earth?

A

95%

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21
Q

How is silicosis characterized?

A

By small rounded modules scattered throughout the lungs.

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22
Q

No single nodule is greater than how many millimeters in diameter?

A

9 mm

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23
Q

T or F; Patient with a simple silicosis are usually symptom-free?

A

True

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24
Q

T or F; Complicated silicosis is characterized by nodules that coalesce and form large masses if in tissue usually in the upper lobes and perihilar regions?

A

True

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25
Q

What is beryllium?

A

A steel gray lightweight metal found in certain plastics, ceramics, rocket fuel, and x-ray tubes?

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26
Q

When beryllium is inhaled, the fumes or particles may cause what?

A

A toxic or allergic pneumonitis, sometimes accompanied by rhinitis, pharyngitis and tracheobronchitis

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27
Q

How is the more complex form of berylliosis characterized?

A

By the development of granulomas, and a diffuse interstitial inflammatory reaction

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28
Q

What is hypersensitivity pneumonitis?

A

A cell mediated immune response of the lungs caused by the inhalation of a variety of offending agents or antigens

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29
Q

What is another name for hypersensitivity pneumonitis?

A

Allergic alveolitis or extrinsic allergic alveolitis

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30
Q

What do the antigens contain in hypersensitivity pneumonitis?

A

Grains, silage, bird, droppings, feathers, wood, dust, cork dust, animal pelts, coffee, beans, fish, meal mushroom, compost, and molds that grow on sugarcane barley and straw

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31
Q

The lung inflammation, or pneumonitis develops, after repeated and prolonged exposure to the allergen. true or false?

A

True

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32
Q

Hypersensitivity pneumonitis caused by the inhalation of moldy hay is called what?

A

Farmers lung

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33
Q

What is the major organ affected by the side effects of medications and illicit drugs?

A

The lungs

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34
Q

What is the largest group of agents associated with interstitial lung disease?

A

Chemotherapeutics

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35
Q

Drug induced interstitial disease may be seen as early as how long after exposure to these agents?

A

One month to as late as several years

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36
Q

What is the precise cause of drug induced interstitial lung disease?

A

It is not known

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37
Q

What are the two phases of radiation therapy?

A

Acute pneumatic phase and late fibrotic phase

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38
Q

Acute pneumonitis is rarely seen in patients who receive a total radiation dose of less than how many rad?

A

3500

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39
Q

What is the amount of drug exposure that will cause ILD near and in the radiated areas?

A

6000 rad over 6 weeks

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40
Q

How long does the acute pneumonitic phase develop after exposure?

A

2 to 3 months

41
Q

The late phase of fibrosis may develop after what:

A
  1. Immediately after the development of acute pneumonitis.
  2. Without an acute pneumonotic period
  3. After a symptom free latent period
42
Q

What is the precise cause of radiation therapy?

A

Not known

43
Q

What is scleroderma?

A

characterized by chronic hardening and thickening of the skin caused by new collagen formation.

44
Q

How may scleroderma occur?

A

It may occur in a localized form or as a systemic disorder (called systemic sclerosis).

45
Q

What is Progressive systemic sclerosis (PSS)?

A

is a relatively rare autoimmune disorder that
affects the blood vessels and connective tissue. It causes fibrous degeneration of the connective tissue of the skin, lungs, and internal organs, especially the esophagus, digestive tract, and kidney.

46
Q

What can scleroderma cause?

A

Pulmonary fibrosis

47
Q

Scleroderma is seen in women in what age?

A

30-50

48
Q

What is Rheumatoid arthritis?

A

Inflammatory joint disease

49
Q

What will rheumatoid arthritis involve?

A

(1) pleurisy, with or without effusion; (2) interstitial pneumonitis; (3) necrobiotic nodules, with or without cavities; (4) Caplan’s syndrome; and (5) pulmonary hypertension secondary to pulmonary vasculitis.

50
Q

What is the most common pulmonary complication associated with rheumatoid arthritis?

A

Pleurisy with or without effusion

51
Q

Who develops rheumatoid arthritis the most men or women?

A

Men

52
Q

When present where would you see rheumatoid arthritis?

A

Unilaterally on the right side

53
Q

How is RA characterized?

A

by alveolar wall fibrosis, interstitial and intraalveolar mononuclear cell infiltration, and lymphoid nodules.

54
Q

In severe cases of RA what can happen?

A

extensive fibrosing alveolitis and honeycombing may develop.

55
Q

What are necrobiotic molecules?

A

gradual degeneration and swelling of lung tissue.

56
Q

What is caplans syndrome (rheumatoid pneumoconiosis)?

A

a progressive pulmonary fibrosis of the lung com- monly seen in coal miners. Caplan’s syndrome is characterized by rounded densities in the lung periphery that often undergo cavity formation and, in some cases, calcification.

57
Q

What is a common secondary complication caused by the progression of fibrosing alveolitis and pulmonary vasculitis.

A

PH

58
Q

What is Sjögren’s Syndrome?

A

a lymphocytic infiltration that primarily involves the salivary and lacrimal glands and is manifested by dry mucous membranes, usually of the mouth and eyes.

59
Q

What does SS include?

A

1) pleurisy with or without effusion, (2) interstitial fibrosis that is indistinguishable from that of other collagen vascular disor- ders, and (3) infiltration of lymphocytes of the tracheobron- chial mucous glands, which in turn causes atrophy of the mucous glands, mucous plugging, atelectasis, and secondary infections.

60
Q

Who is Ss most commonly seen in?

A

Women

61
Q

What percentage of people get RA from SS.

A

90%

62
Q

What percentage get SS?

A

50%

63
Q

What is polymyositis dermatitis?

A

A diffuse inflammatory disorder of the striated muscles that primarily weakens the limbs, neck, and pharynx.

64
Q

What is dermatomytosis?

A

When the erythematous skin rash accompanies the muscle weakness

65
Q

Pulmonary involvement develops in response to:

A
  1. Recurrent episodes of aspiration pneumonia caused by esophageal weakness and atrophy
  2. Hypostatic pneumonia secondary to weakened diaphragm.
  3. Drug induced interstitial pneumonitis
66
Q

Is PD seen more in women or men? What is the ratio?

A

Women; 2:1

67
Q

What 2 age groups does PD usually occur in?

A

Before the age of 10 & from 40 to 50 years

68
Q

What percentage of patients show your pulmonary manifestations in 1-24 months before the striated muscle or skin shows signs or symptoms

A

40%

69
Q

What is SLE?

A

A multisystem disorder that mainly involves the joints and skin.

70
Q

Involvement of the lungs appears in about what percent of the stages?

A

50-70%

71
Q

Pulmonary manifestations for SLE are characterized by what?

A
  1. Pleurisy with or without perfusion
  2. Atelectasis
  3. Diffuse infiltrates and pneumonitis
  4. Diffuse ILD
  5. Uremic pulmonary edema
  6. Diaphragmatic dysfunction
  7. Infections
72
Q

What is sarcoidosis?

A

Chronic disorder of unknown origin. Characterized by the formation of tubercular and nonnecratizing epitheliod tissue.

73
Q

What some common sites for sarcoidosis?

A

Eyes, lung, spleen, liver, skin, mucous membranes, salivary glands (with involvement of lymph glands).

74
Q

What 3 immunoglobins will you see and increase in for sarcoidosis?

A

IgM, IgG, IgA

75
Q

What will you see and elevation of for sarcoidosis?

A

Angiotensin converting enzyme

76
Q

What ethnicity is sarcoidosis most common among?

A

African Americans

77
Q

What age does it appear most frequently?

A

10-40 with highest incident of 20-30

78
Q

What gender is most affected?

A

Women

79
Q

What is IPF?

A

A progressive inflammatory disease with varying degrees of fibrosis and in severe cases honeycombing.

80
Q

What gender and age is IPF most commonly seen?

A

Men ages 40-70

81
Q

When does death usually occur?

A

4-10 years

82
Q

What is COP A.k.a BOOP?

A

Connective tissue plugs in the small airways & mononuclear cell infiltration of the surrounding parenchyma.

83
Q

What is LIP?

A

Fibrosis and accumulation of lymphocytes in the lungs

84
Q

What is LAM?

A

A rare lung disease involving the smooth muscle of the airways and affects women of childbearing age

85
Q

What is PLCH?

A

A smoking related disease characterized by by mid lung zone star shaped nodules with adjacent thin walled cysts.

86
Q

What is alveolar protienosis?

A

When the alveoli fill with protein

87
Q

What is the diagnosis for proteinosis

A

Transbronchial or open lung biopsy, and bronchial lavage.

88
Q

What are pulmonary vasculitides?

A

Inflammation and destruction of pulmonary vessels.

89
Q

What is wegener granulomatosis?

A

Multi system disorder characterized by:
1. Necrotizing granulomatus vascultis
2. Focal and segmental glomerulonephritis
3. Variable degrees of systemic vasculitis in small arteries and veins

90
Q

Who is it most commonly seen in?

A

Men over the age of 50

91
Q

What is the tx?

A

Open lung biopsy

92
Q

What is CSS?

A

A necrotizing vasculitis that predominantly involves the small vessels of the lungs. Characterized by heavy infiltrate if eosinophils.

93
Q

What is LG?

A

Necrotizing vasculitis that involves the lungs lesions are in lower lobes and cavities develop in more that 1/3 of cases.

94
Q

What is good pasture syndrome?

A

Involves the lungs and the kidneys and a recurrent episodes of pulmonary hemmorhage, hemoptysis and pulmonary fibrosis.

95
Q

What is the survival rate for good pasture syndrome?

A

80%

96
Q

Good pasture syndrome will demonstrate an increase DLCO?

A

True

97
Q

What is chronic eosinophillic pneumonia?

A

Infiltration of eosinophils

98
Q

What are ways to manage ILD?

A

Corticosteroids
immunosuppressant agents
Plasmapheresis
bronchial lavage

99
Q

What are the protocols?

A

O2 therapy
Mechanical ventilation