Diffuse Interstitial Disease Flashcards

1
Q

A group of chronic disorders characterized by inflammation and fibrosis of the pulmonary interstitial tissue

A

diffuse interstitial disease

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2
Q

Known collectively as restrictive lung disease

A

diffuse interstitial disease

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3
Q

What are diffuse interstitial diseases caused by?

A

Many causes, poorly understood

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4
Q

Diffuse interstitial diseases are responsible for about __% of __________ pulmonary diseases.

A

Diffuse interstitial diseases are responsible for about 15% of noninfectious pulmonary diseases.

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5
Q

What are some fibrosing diseases? (7)

A
  • Idiopathic pulmonary fibrosis
  • Nonspecific interstitial pneumonia
  • Cryptogenic organizing pneumonia
  • Pulmonary fibrosis associated with connective tissue diseases
  • Pneumoconiosis
  • Drug reactions
  • Radiation pneumonitis
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6
Q

What is a granumlomatous disease?

A

tuberculosis

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7
Q

What are types of diffuse interstitial disease?

A
  • fibrosing dz
  • granulomatous dz
  • pulmonary eosinophilia
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8
Q

What is the pathogenesis of interstitial fibrosis?

A
  1. endothelial damage and activation
  2. fibroblast migration and proliferation, basement membrane disruption
  3. angiogenesis and myofibroblast foci formulation
  4. pulmonary fibrosis
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9
Q

chronic process of pulmonary fibrosis or scarring due to unknown causes

A

idiopathic pulmonary fibrosis

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10
Q

What is idiopathic pulmonary fibrosis cause by?

A

Caused by recurrent inflammation and repair in susceptible persons

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11
Q

With IPF, what is the cause the susceptible persons?

A

hereditary

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12
Q

What does IPF result in?

A

decreased lung compliance

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13
Q

What will IPF show with spirometry

A

decreased lung volume

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14
Q

What is IPF associated with?

A
  • pulmonary HTN

- right HF

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15
Q

Does IPF occur abruptly or insidiously?

A

insidiously

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16
Q

What are the initial symptoms of IPF?

A

gradually increasing dyspnea on exertion and dry cough

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17
Q

What are the later symptoms of IPF?

A
  • hypoxemia
  • cyanosis
  • clubbing
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18
Q

What is the typical age range for presentation with IPF?

A

40-70 yo

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19
Q

What are some general symptoms of IPF? (5)

A
  • Shortness of breath
  • Chronic dry, hacking coughing
  • Fatigue and weakness
  • Chest discomfort
  • Loss of appetite and rapid weight loss
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20
Q

What are some CXR features of IPF?

A
  • Reduced lung volume
  • Reticular opacities
  • Lower lobe and peripheral field predominance
  • Honeycombing may be visible (fibrocystic appearance)
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21
Q

What imaging technique can you best se honeycombing?

A

better on CT versus xray

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22
Q

diseases that can involve the lung, resulting in different presentations including pulmonary interstitial fibrosis (other manifestations include vascular sclerosis, pneumonia, and bronchiolitis)

A

autoimmune connective tissue diseases

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23
Q

What are autoimmune connective tissue diseases associated with?

A
  • pulmonary hypertension

- right HF

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24
Q

What are the causes of autoimmune connective tissues diseases? (3)

A
  • Rheumatoid arthritis
  • Systemic sclerosis (scleroderma)
  • Lupus erythematosus
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25
Q

What percent of patients is rheumatoid arthritis the cause of autoimmune connective tissue diseases?

A

30-40%

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26
Q

Lung fibrosis in response to inhaled particles and aerosols

A

pneumoconiosis

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27
Q

With pneumoconiosis, ____________ ingest particles, die and release ________ contents and cytokines causing _______ without clearing ________ stimulus.

A

With pneumoconiosis, macrophages ingest particles, die and release lysosomal contents and cytokines causing fibrosis without clearing inflammatory stimulus.

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28
Q

What size are particles that are most likely to reach terminal alveoli?

A

1-5 microns

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29
Q

Pneumoconiosis has a similar pathology as which condition?

A

IPF

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30
Q

What are the causes of pneumoconiosis?

A
  • mineral dust
  • organic dust
  • chemical fumes
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31
Q

What are some examples of mineral dust?

A
  • coal
  • silica
  • asbestos
  • iron oxide
32
Q

What are organic dusts also associated with?

A
  • hypersensitivity pneumonitis

- asthma

33
Q

What are some examples of hypersensitivity pneumonitis causes?

A
  • moldy hay

- bird droppings

34
Q

What are examples of asthma causes?

A
  • cotton
  • flax
  • hemp
  • red cedar dust
35
Q

What are some examples of chemical fumes?

A
  • nitrous oxide
  • sulfur dioxide
  • ammonia
  • benzene
  • insecticides
36
Q

What describes when certain drugs may be associated with acute and chronic alterations in respiratory structure and function?

A

drug-induced pulmonary disease

37
Q

Drug-induced pulmonary disease has similar process as what condition?

A

IPF

38
Q

What are some drugs that can cause drug-induced pulmonary disease? (6)

A
  • bleomycin
  • methotrexate
  • amiodarone
  • nitrofurantoin
  • aspirin
  • beta antagonists
39
Q

What are 2 examples of granulomatous diseases?

A
  • sarcoidosis

- hypersensitivity pneumonitis

40
Q

a firm nodule resulting from chronic inflammation, often with a fibrous capsule

A

granuloma

41
Q

What does a granuloma contain?

A

-macrophages
-lymphocytes
-plasma cells
-multinucleated giant cells
(all of these are associated with chronic inflammation)

42
Q

Granulomas may contain ______ organisms and may have central ______.

A

Granulomas may contain infectious organisms and may have central necrosis.

43
Q

What organism is caseating associated with specifically?

A

mycobacterium

44
Q

What are examples of infectious causes of granulomatous diseases?

A
  • bacteria
  • fungi
  • parasites
45
Q

What are examples of noninfectious causes of granulomatous diseases?

A
  • sarcoidosis

- hypersensitivity pneumonitis

46
Q

An immunologically-mediated systemic disease of unknown cause characterized by non-caseating granulomas in many tissues and organs

A

sarcoidosis

47
Q

What organs and tissues are most commonly affected by sarcoidosis?

A
  • lungs
  • LN/spleen
  • eyes
  • skin
48
Q

How do you differentiate sarcoidosis from infectious causes?

A

biopsy will show an absence of microbes

49
Q

What are CXR findings of sarcoidosis?

A
  • bilateral hilar lymph node enlargement

- milia-size and larger granulomas that may cavitate

50
Q

What percentage of sarcoidosis cases will you see bilateral hilar lymph node enlargement?

A

90% of cases

51
Q

What may the granulomas in the lung initiate?

A

initiate interstitial fibrosis and pulmonary edema

52
Q

What are some other signs of sarcoidosis?

A
  • skin granulomas

- eye granulomas

53
Q

immunologically-mediated inflammatory lung disorders caused by intense, usually prolonged exposure to inhaled animal, microbial, and some chemical antigens

A

hypersensitivity pneumonitis

54
Q

What does hypersensitivity pneumonitis causes diffuse inflammation of and what can that result in?

A

Causes diffuse inflammation of interstitial lung, terminal bronchioli, and alveoli, can result in fibrosis

55
Q

What does hypersensitivity pneumonitis require?

A

Requires abnormal sensitivity or heightened reactivity to the antigen = susceptible individuals

56
Q

What are the 2 primary types of hypersensitivity reactions that cause hypersensitivity pneumonitis?

A
  • type 3 (immune complex)

- type 4 (delayed hypersensitivity)

57
Q

Acute attacks, which follow inhalation of antigenic dust in sensitized patients

A

hypersensitivity pneumonitis

58
Q

With hypersensitivity pneumonitis, what are there recurring episodes of?

A
  • fever
  • dyspnea
  • cough
  • leukocytosis
59
Q

with hypersensitivity pneumonitis, when do symptoms usually appear?

A

usually appear 4-6 hours after exposure

60
Q

What may hypersensitivity pneumonitis progress to?

A

may progress to:

  • respiratory failure
  • dyspnea
  • cyanosis
  • decrease in total lung capacity and compliance
61
Q

How can hypersensitivity pneumonitis be prevented?

A

Can be prevented by removal of the stimulus, if recognized early

62
Q

What are the common sensitizing agents for hypersensitivity pneumonitis?

A

Common sensitizing agents include:

  • organic dust with bacterial spores
  • fungi
  • animal proteins
  • bacterial products
63
Q

results from exposure to dusts generated from harvested humid, warm hay that permits the rapid proliferation of the spores of thermophilic actinomycetes

A

Farmer’s lung

64
Q

What are the spores associated with Farmer’s lung?

A

thermophilic actinomycetes

65
Q

provoked by proteins from serum, excreta, or feathers of birds

A

bird breeder’s lung/bird fancier’s disease

66
Q

caused by thermophilic bacteria in heated water reservoirs

A

Humidifier or air-conditioner lung

67
Q

What are CXR findings for hypersensitivity pneumonitis?

A

diffuse and nodular infiltrates

68
Q

Several pulmonary disorders characterized by an infiltration of eosinophils into alveoli, resulting in intraseptal inflammation, fibrosis

A

pulmonary eosinophilia

69
Q

What does pulmonary eosinophilia result in?

A
  • intraseptal inflammation

- fibrosis

70
Q

What is the cause of pulmonary eosinophilia?

A

frequently idiopathic

71
Q

Assessment of diffuse interstitial disease

A
  • History and physical examination
  • Spirometry
  • CXR or CT
  • Oximetry, arterial blood gas, acid-base balance
  • Endoscopic bronchial biopsy
  • Laboratory assessment for autoimmune diseases (rheumatoid arthritis, lupus erythematosus, scleroderma, sarcoidosis)
72
Q

What are symptoms of diffuse interstitial disease?

A
  • dyspnea

- tachypnea

73
Q

What are signs of diffuse interstitial disease?

A
  • End-inspiratory crackles (without wheezing or other evidence of airway obstruction)
  • Cyanosis
  • Increased blood CO2 (hypercapnia) and reduced O2 (hypoxia)
  • Reduced lung volume (FVC, TLC) and compliance (FEV1) by spirometry
74
Q

What are CXR findings for diffuse interstitial disease?

A

CXR shows bilateral small nodules, irregular lines, ground-glass shadows

75
Q

What does spirometry show with diffuse interstitial disease?

A
  • Reduced lung volume (FVC, TLC)

- Reduced compliance (FEV1)

76
Q

What does diffuse interstitial disease frequently result in?

A

Frequently results in:

  • secondary pulmonary hypertension
  • cor pulmonale
77
Q

Why are end-stage diffuse interstitial diseases hard to differentiate?

A

hard to differentiate because they result in similar scarring and destruction of the lung (called end-stage lung or honeycomb lung)