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Cadiopulmonary Medicine > Test 5 Ch. 27 Interstitial Lung Diseases > Flashcards

Test 5 Ch. 27 Interstitial Lung Diseases Flashcards

1
Q

This refers to a broad group of inflammatory lung disorders

A

interstitial lung disease (ILD)

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2
Q

ILD is also called (3)

A
  • diffuse interstitial lung disease
  • fibrotic interstitial lung disease
  • pulmonary fibrosis
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3
Q

More than _____ disease entities are characterized by _____, ________, or _______ inflammatory of the alveolar walls by cells, fluid and connective tissue

A

180
acute
subacute
chronic

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4
Q

If ILD is left untreated the inflammatory process can progress to

A

irreversible pulmonary fibrosis

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5
Q

The anatomic alterations of ILD may involve the (3)

A
  • bronchi
  • alveolar walls
  • adjacent alveolar spaces
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6
Q

In severe cases the extensive inflammation leads to…(4)

A
  • pulmonary fibrosis
  • granulomas
  • honeycombing
  • cavitation
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7
Q

During this stage of any ILD, the general inflammatory condition is characterized by edema and the infiltration of a variety of WBC in the alveolar walls and interstitial spaces

A

Acute stage

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8
Q

This stage may be followed by future interstitial thickening, fibrosis, granulomas, and in some cases, honeycombing and cavity formation. Pleural effusion may be present

A

Chronic stage

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9
Q

Interstitial lung disorders produce a

A

restrictive lung conditions

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10
Q

Pathologic and structural associated w/ chronic ILD: (only 1 that does not have atelectasis)

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
  • Bronchospasm
  • Excessive bronchial secretions
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11
Q

B/c there are more than 180 different pulmonary disorders classified as ILD, it is helpful to group them according to (3)

A
  • their occupation or environmental exposure
  • disease associations
  • specific pathology
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12
Q

Occupation/ Environment : Inorganic Substance Exposure

A
  • Asbestosis
  • Coal dust
  • Silica
  • Beryllium
  • Aluminum
  • Barrium
  • Clay
  • Iron
  • Certain talcs
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13
Q

Occupation/ Environment: Organic Exposure

A
  • Hypersensitivity pneumonitis
  • Moldy hay
  • Silage
  • Modly sugar cane
  • Mushroom compost
  • Barley
  • Cheese
  • Wood pulp, bark, dust
  • Cork dust
  • Bird droppings
  • Paintis
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14
Q

Occupation/ Environment: Medication: illicit drugs

A
  • Antibiotics
  • Anti-inflammatory agents
  • Cardiovascular agents
  • Chemotherapeutic agents
  • Drug-induced systemic lupus erythemaatosus
  • illicit drugs
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15
Q

Occupation/ Environment: other

A
  • Radiation
  • Irritant gases
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16
Q

Exposure to asbestos may cause ____________, a form of ILD

A

asbestosis

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17
Q

There are two primary types of asbestos:

A
  1. amphiboles (crocidolite, amosite, anthophyllite)
  2. chrysotile (most commonly used in industry)
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18
Q

Asbestos fibers typically range from __ to ___ in length and are about ___ in diameter

A

50 to 100;
0.5 um

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19
Q

The ____________ have the longest and strongest ___________.

A

chrysotiles;
fibers

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20
Q

What is most common in diagnosis in pts with an asbestos exposure history

A

Pleural calcification

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21
Q

The pulmonary deposition and accumulation of large amounts of coal dust cause

A

coal worker pneumoconiosis (CWP)

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22
Q

CWP is also known as

A

coal miner lung and black lung

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23
Q

Simple CWP is characterized by the presence of

A

pinpoint nodules called coal macules (black spots)

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24
Q

The coal macules often develop around _____ and _________- generation respiratory bronchioles and cause adjacent alveoli to retract, which is called ________ emphysema

A

first and second;
focal

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25
Q

This is characterized by areas of fibrotic nodules greater than 1 cm in diameter

A

progressive massive fibrosis (PMF)

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26
Q

The fibrotic changes in CWP are usually caused by _______

A

silica

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27
Q

This is caused by the chronic inhalation of crystalline, free silica, or silicon dioxide particles

A

Silicosis

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28
Q

Silica is the main component of more than % of the rocks of the earth

A

95

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29
Q

Simple silicosis is characterized by…….. scattered through the lungs

A

small rounded nodules

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30
Q

No single nodule is greater than

A

9 mm in diameter

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31
Q

Pts w/ silicosis are usually

A

symptom- free

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32
Q

This is characterized by nodules that coalesce and form large masses of fibrous tissue, usually in the upper lobes and perihilar regions

A

Complicated silicosis

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33
Q

In severe cases of complicated silicosis the fibrotic regions may undergo

A

tissue necrosis and cavitate

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34
Q

_____________ is a steel-gray, lightweight metal found in certain plastics and ceramics, rocket fuels and xray tubes

A

Beryllium

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35
Q

This is characterized by the development of granulomas and a diffuse interstitial inflammatory reaction

A

The more complex form of beryllium

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36
Q

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

A

Hypersensitivity pneumonitis

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37
Q

Other names for Hypersensitivity Pneumonitis (2)

A
  • allergic alveolitis
  • extrinsic allergic
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38
Q

Hypersensitivity Pneumonitis antigens include

A
  • grains
  • silage
  • bird droppings
  • feathers
  • wood dust (redwood and maple)
  • cork
  • dust
  • animal pelts
  • coffee beans
  • fish meal
  • mushroom compost
  • mold that grows on sugar cane
  • barley
  • straw
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39
Q

The inhalation of moldy hay causes

A

farmer’s lung

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40
Q

What are by far the largest group of agents associated with ILD

A

chemotherapeutics (anticancer agents)

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41
Q

What are the names of the major offenders for anticancer agents

A
  • Bleomycin
  • mitomycin
  • busulfan
  • cyclophosphamide
  • methotrexate
  • carmustine
    BCNU
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42
Q

An antibacterial drug used in the treatment of urinary tract infections is associated with ILD

A

Nitrofurantoin

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43
Q

The excessive long-term administration of oxygen (oxygen toxicity) is known to cause diffuse

A

pulmonary injury and fibrosis

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44
Q

What is the cause of drug-induced ILD?

A

it is unknown

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45
Q

What is the diagnosis confirmed by in drug induced ILD?

A

open lung biopsy

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46
Q

____________ therapy is the management of cancer may cause ILD

A

Radiation

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47
Q

Radiation-induced lung disease is commonly divided into two major phases:

A
  • acute pneumonitic phase
  • late fibrotic phase
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48
Q

Is rarely seen in pts who receive a total of less than 3500 rad, but if doses in excessive 6000 rad, over 6 weeks almost always causes ILD, near the radiated areas

A

Acute pneumonitic

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49
Q

The acute pneumonitic phase develops about ___ to __ months after exposure

A

2 to 3

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50
Q

The late phase of fibrosis may develop (3)

A
  1. immediately
  2. w/o an acute pneumonitic period
  3. after symptom free latent period
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51
Q

When fibrosis does develop it generally happens after ___ to ____ months after radiation exposure

A

6 to 12

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52
Q

What is often associated with the late fibrotic phase?

A

Pleural effusion

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53
Q

The precise cause of radiation therapy is

A

unknown

54
Q

radiation- therapy is diagnosed by (2)

A
  • history of recent radiation
  • open lung biopsy
55
Q

The inhalation of irritant gases may cause

A

acute chemical pneumonitis and in severe cases ILD

56
Q

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

A

Scleroderma

57
Q

A rare autoimmune disorder that affects the blood vessels and connective tissue

A

Progressive systemic sclerosis (PSS)

58
Q

PSS causes fibrosis degeneration of the connective tissue of the

A

skin, lungs, and internal organs, especially the esophagus, digestive tract, and kidney

59
Q

Scleroderma is most commonly seen in _________ ages……

A

women 30 to 50 y/o

60
Q

This is an inflammatory joint disease

A

Rheumatoid Arthritis

61
Q

Rheumatoid Arthritis may involve the lungs in the form of (5)

A
  1. Pleurisy w/ or w/o effusion
  2. interstitial pneumonitis
  3. necrobiotic nodules w/ or w/o cavities
  4. Caplan syndrome
  5. pulmonary hypertension secondary to pulmonary vasculitis
62
Q

What is the most common Pulmonary complication associated w/ rheumatoid arthritis

A

pleurisy

63
Q

Who develops rheumatoid pleural complication

A

men

64
Q

Rheumatoid interstitial pneumonitis is characterized by (3)

A
  • alveolar wall fibrosis
  • interstitial and intraalveolar mononuclear cell infiltration
  • lymphoid nodules
65
Q

This is progressive pulmonary fibrosis of the lung commonly seen in coal miners

A

Caplan syndrome

66
Q

Caplan syndrome is characterized by ___________ ________ in the lung periphery that often undergoes ________ _____________ and in some cases calcification

A

rounded densities
cavity formation

67
Q

This is a lymphocytic infiltration that primarily involves the salivary and lacrimal (eyes) glands and manifests by dry mucous membranes, usually the mouth an eyes

A

Sjogren Syndrome

68
Q

Pulmonary involvement frequently occurs in Sjögren syndrome includes (3)

A
  1. pleurisy w/ or w/o effusion
  2. interstitial fibrosis that is indistinguishable from that of other collagen vascular disorders
  3. infiltration of the lymphocytes, low lymphocytes the TB tree mucus glands, mucous plugging, atelectasis and secondary infections
69
Q

Sjögren syndrome occurs most often in……..
And is commonly associated w/ rheumatoid arthritis (___% of pts w/ Sjögren syndrome)

A

women (90%);
50%

70
Q

___________________ is a diffuse inflammatory disorder of the striated muscles that primarily weakens the limbs, neck and pharynx

A

Polymyositis

71
Q

When erythematous skin rash accompanies the muscle weakness. What is this called

A

Dermatomyositis

72
Q

Pulmonary involvement develops in response to

A
  1. recurrent episodes of aspiration PNA caused by esophageal weakness and atrophy
  2. hypostatic PNA secondary to a weakened diaphragm
  3. drug-induced interstitial pneumonitis
73
Q

Polymyositis-dermatomyositis is seen more often in…….. at about ___ ratio

A

women then men ; 2:1 ratio

74
Q

This a multi-system disorder that mainly involves the joints and skin, it can also cause damage to kidneys, lungs, nervous system and heart

A

Systemic lupus erythematosus (SLE)

75
Q

Involvement of the lungs appears in SLE about

A

50% to 70% of cases

76
Q

Pulmonary manifestations are characterized by

A
  1. Pleurisy w/ or w/o effusion
  2. atelectasis
  3. diffuse infiltrates and pneumonitis
  4. diffuse ILD
  5. uremic pulmonary edema
  6. diaphragmatic dysfunction
  7. infections
77
Q

What is the most common pulmonary complication of SLE?

A

Pleurisy w/ or w/o effusion

78
Q

A common chronic disorder of unknown origin characterized by the formation of tubercles of non necrotizing epithelial tissue (non casting granulomas)

A

Sarcoidosis

79
Q

Common sites for sarcoidosis are:

A
  • eyes
  • lungs
  • spleen
  • liver
  • skin
  • mucous membranes
  • lacrimal and salivary glands
80
Q

One of the clinical laboratory hallmarks of sarcoidosis is an increase in

A

all three immunoglobulin, IgM, IgG, IgA

81
Q

Sarcoidosis is more common in

A

African America

82
Q

Who is affected more by sarcoidosis

A

Women

83
Q

Is progressive and inflammatory disease w/ varying degrees of fibrosis and in severe cases honeycombing

A

Idiopathic pulmonary fibrosis (IPF)

84
Q

IPF is more chronic and death usually occurs in ___ to ___ years

A

4 to 10

85
Q

This is characterized by connective tissue plugs in the small airways and mononuclear cell infiltration of the surrounding parenchyma

A

Cryptogenic organizing PNA (COP) or Bronchiolitis obliterans organizing PNA (BOOP)

86
Q

COP is idiopathic and is one of the ILD in which both

A

restrictive and obstructive findings

87
Q

This is a diffuse pulmonary disorder characterized by fibrosis and accumulation of lymphocytes in the lungs

A

Lymphocytic interstitial PNA (LIP)

88
Q

LIP is commonly associated with

A

lymphoma

89
Q

The diagnosis of LIP requires a

A

surgical lung biopsy

90
Q

This is a rare lung disease involving the smooth muscles of the airways and affects women of childbearing age

A

Lymphangioleiomyomatosis (LAM)

91
Q

The diagnosis of LAM is confirmed w/ an

A

open lung biopsy

92
Q

LAM’s disease slowly progresses over ___ to ___ years, ending in ______ resulting from ______________ _____________.

A

2 to 10 years;
death;
ventilatory failure

93
Q

This is a smoking-related ILD characterized by middling zone star-shaped nodules w/ adjacent thin-walled cysts

A

Pulmonary Langerhans Cell Histiocytosis (PLCH)

94
Q

Diagnosis is confirmed histologiically by

A

tissue biopsy

95
Q

This is a condition of unknown cause in which the alveoli become filled w/ protein and lipids

A

Pulmonary Alveolar Proteinosis (PAP)

96
Q

What does the chest xray typically reveals in PAP

A

Bilateral infiltrates, prominent in the perihilar regions (butterfly pattern)

97
Q

What are the 2 diagnosis for PAP

A
  • transbronchial or open lung biopsy
  • Analysis of fluid removed during WLL
98
Q

This is characterized by inflammation and destruction of the pulmonary vessels

A

Pulmonary Vasculitides

99
Q

The major categories in pulmonary vasculitides are: (3)

A
  • Wegner granulomatosis
  • Churg- Strauss syndrome
  • lymphomatous granulomatosis
100
Q

This is a disease of unknown cause that involves two organ systems: kidneys and lungs

A

Goodpasture syndrome

101
Q

In the lungs recurrent episodes of

A

pulmonary hemorrhage and hemoptysis and in some cases pulmonary fibrosis

102
Q

In the kidneys there is a glomerulonephritis characterized by infiltration of antibodies within the

A

glomerular basement membrane (GBM)

103
Q

Goodpasture disease is usually seen in

A

young adults

104
Q

With appropriate tx, the __ year survival rate is almost ___%

A

5;
80%

105
Q

YELLOW PAGES
What are the clinical manifestations for ILD

A
  • increased AC membrane thickness
  • excessive bronchial secretions
106
Q

Vital signsof ILD (3)

A
  • Increased RR
  • Increased HR
  • Increased BP
107
Q

Chest Assessment Findings for ILD: (6)

A
  • increased tactile and vocal fremitus
  • Dull percussion note
  • Bronchial breath sounds
  • Crackles
  • Pleural friction rub
  • Whispered pectoriliquy
108
Q

Lab Findings in ILD

A

Increased hematocrit and hemoglobin (polycythemia)

109
Q

Chest Xray in ILD

A
  • Bilateral reticulondular
  • Irregularly shaped opacities
  • Granulomas
  • Cavity formation
  • Honeycombing
  • Pleural effusion
  • Pleural thickening
110
Q

Which disease process demonstrates an increased pulmonary diffusion capacity (DLCO)

A
  • Goodpasture syndrome
  • Idiopathic Pulmonary Hemosiderosis
111
Q

This is an entity of unknown cause that is characterized by recurrent episodes of pulmonary hemorrhage similar seen in Goodpasture syndrome

A

Idiopathic Pulmonary Hemosiderosis

112
Q

This is characterized by infiltration of eosinophils and lesser extent macrophages into the alveolar and interstitial spaces

A

Chronic Eosinophilic PNA

113
Q

Diagnosis of chronic esinophillic PNA

A

open lung biopsy

114
Q

General management for ILD: MEDs 2

A
  • Corticosteroids
  • Immunosuppressive Agents
115
Q

What the Respiratory Protocols for ILD (2)

A
  • Oxygen Therapy
  • Mechanical Ventilation
116
Q

What is the tx for Goodpasture syndrome?

A

Plasmapheresis, which removes the anti-GBM antibodies

117
Q

What is the tx for PAP

A
  • WLL
  • Bronchial lavage
118
Q

Questions from the back
Which of the following is another name for hypersensitivity pneumonitis

A

Extrinsic allergic alveolitis

119
Q

Which of the following are considered pulmonary vasculitides

A
  • Wegener granulomatosis
  • Lymphomatoid granulomatosis
  • Churg- Strauss syndrome
120
Q

Which asbestos is most commonly used in the industry?

A

chrysotile

121
Q

What is another name amphiboles (a type of asbestoses) (3)

A
  • crocidolite
  • amosite
  • anthophyllite
122
Q

Which of the following disorders is associated with desquamative interstitial PNA and unusual interstitial PNA

A

Idiopathic pulmonary fibrosis

123
Q

Which of the following are systemic connective tissue disease

A
  • Rheumatoid Arthritis
  • Sjögren syndrome
124
Q

Which of the following pulmonary function study findings are associated with chronic ILD

A
  • Decreased FEVT
  • Decreased FVC
125
Q

Which of the following hemodynamic indices are associated w/ advance or severe ILD

A
  • Increased CVP
  • Increased PA
126
Q

Which of the following chest assessment findings is associated w ILD

A
  • Bronchial breath sounds
127
Q

Which of the following oxygenation indices are associated with pneumoconiosis

A
  • Increased O2ER
  • Decreased SvO2
128
Q

The fibrotic changes that develop in coal work pneumoconiosis usually result from

A
  • silica
129
Q

Which of the following g is associated with ILD

A
  • pleural friction rub
  • dull percussion note
  • cor pulmonale
  • Elveated PA
130
Q

*YELLOW PAGES CONT.**
Inspection of ILD (4)

A
  • Cyanosis
  • Digital Clubbing
  • Peripheral Edema and Venous Distention
  • Nonproductive cough
131
Q

Because polycythemia and cor pulmonale are associated with chronic ILD, the following may occur: (3)

A
  • Distended neck veins
  • pitting edema
  • Enlarged and tender liver

Cadiopulmonary Medicine (24 decks)

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