Respiratory Path 3 - Galbraith Flashcards

1
Q

Inflammation and pulmonary interstitial tissue fibrosis, especially in the alveolar walls

A

Chronic Diffuse Interstitial (Restrictive) Disease

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2
Q

Interstitial fibrosis leads to what 3 things?

A
  • Low diffusing capacity
  • Low lung volume
  • Low lung compliance
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3
Q

Honeycomb lung

A

Advanced stage extensive scarring in any of the restrictive lung disorders

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4
Q

Progressive interstitial fibrosis and respiratory failure, unknown cause

A

Idiopathic pulmonary fibrosis

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5
Q

Idiopathic pulmonary fibrosis is THOUGHT to occur secondary to _____

A

Repeated alveolar epithelial injury w/ pro-fibrotic response

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6
Q

Causes of repeated alveolar epithelial injury in idiopathic pulmonary fibrosis

A
  • Tobacco smoke
  • Fumes, dusts
  • Viruses
  • Persistent GI reflux
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7
Q

TERT, TERC mutations

A

Telomerase mutations that make for susceptibility to idiopathic pulmonary fibrosis

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8
Q

Molecular cause for the repetitive fibrosis reactions

A

TGF-beta 1

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9
Q
  • Patchy interstitial fibrosis

- New fibroblastic foci mixed with more densely fibrotic areas

A

Usual interstitial pneumonia (UIP) - Morphologic pattern seen in idiopathic pulmonary fibrosis

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10
Q

Spaces surrounded by dense collagen, lined with hyperplastic type 2 pneumocytes and bronchial epithelium

A

Honeycomb fibrosis - common result of restrictive lung diseases

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11
Q

In IPF, the fibrosis is usually in which 2 locations?

A
  • Subpleural

- Interlobular septal

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12
Q

Sign of an early UIP lesion in IPF

A

Cellular fibroblastic foci

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13
Q
  • Gradually worsening dyspnea with exertion
  • Dry cough
  • Late development of progressive hypoxemia, cyanosis, and clubbing
  • Smoker, 40-70
  • Microscopic evidence of fibrosis
A

Idiopathic pulmonary fibrosis

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14
Q

Median survival after diagnosis of IPF is ____

A
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15
Q

Definitive treatment for IPF

A

Lung transplant

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16
Q

How are the microscopic findings in Nonspecific Interstitial Pneumonia (NSIP) different than those in UIP?

A

Develops as EITHER interstitial chronic inflammation OR interstitial fibrosis, NO heterogeneity

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17
Q

Typical person to get NSIP

A

Women, 50s, not a smoker, presents w/ dyspnea and cough

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18
Q

Tufts/balls/plugs of immature fibroblastic tissue found within terminal bronchioles, alveolar ducts, and alveolar spaces

Characteristic of _____

A

Masson bodies

Cryptogenic organizing pneumonia (COP)

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19
Q
  • Cough and dyspnea
  • Patchy subpleural or peribronchial consolidation
  • NO INTERSTITIAL FIBROSIS
  • NO HONEYCOMBING
  • Clusters of fibroblasts/fibrous tissue in terminal airways
  • Normal underlying tissue
A

Cryptogenic organizing pneumonia (COP)

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20
Q

Most cases of COP require ___ for treatment

A

Steroid therapy

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21
Q

Pneumoconioses - general definition

A

Lung diseases due to chronic responses to inhaled aerosols (mineral dusts, organic dusts, chemical fumes, vapors)

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22
Q

Pathogenicity of Pneumoconiosis is determined by what 4 things?

A
  • Particle size (smaller = worse)
  • Particle solubility
  • Level and duration of exposure
  • Intensity of immune response
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23
Q

Black pigmented lesions full of dark macrophages within lymphatic tissues

A

Anthracosis - ASYMPTOMATIC coal worker’s pneumoconiosis

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24
Q

Macules/nodules of dark macrophages AND collagen networks, located adjacent to respiratory bronchioles

A

Simple Coal Worker’s Pneumoconiosis (CWP)

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25
Q

Multiple anthracotic (dark) scars w/ central ischemic necrosis replacing lung parenchyma

A

Progressive massive fibrosis (PMF) - advanced coal workers pneumoconiosis

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26
Q

3 complications of PMF

A
  • Respiratory failure
  • Pulmonary HTN
  • Cor pulmonale
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27
Q

Anthracosis can OCCASIONALLY cause what?

A

Centriacinar emphysema

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28
Q

Anthracosis can OCCASIONALLY cause what?

A

Centriacinar emphysema

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29
Q

Interstitial fibrosis leads to what 2 things?

A
  • Low diffusing capacity
  • Low lung volume
  • Low lung compliance
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30
Q

Honeycomb lung

A

Advanced stage extensive scarring in any of the restrictive lung disorders

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31
Q

Progressive interstitial fibrosis and respiratory failure, unknown cause

A

Idiopathic pulmonary fibrosis

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32
Q

Idiopathic pulmonary fibrosis is THOUGHT to occur secondary to _____

A

Repeated alveolar epithelial injury w/ pro-fibrotic response

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33
Q

Causes of repeated alveolar epithelial injury in idiopathic pulmonary fibrosis

A
  • Tobacco smoke
  • Fumes, dusts
  • Viruses
  • Persistent GI reflux
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34
Q

TERT, TERC mutations

A

Telomerase mutations that make for susceptibility to idiopathic pulmonary fibrosis

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35
Q

Molecular cause for the repetitive fibrosis reactions

A

TGF-beta 1

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36
Q
  • Patchy interstitial fibrosis

- New fibroblastic foci mixed with more densely fibrotic areas

A

Usual interstitial pneumonia (UIP) - Morphologic pattern seen in idiopathic pulmonary fibrosis

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37
Q

Spaces surrounded by dense collagen, lined with hyperplastic type 2 pneumocytes and bronchial epithelium

A

Honeycomb fibrosis - common result of restrictive lung diseases

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38
Q

In IPF, the fibrosis is usually in which 2 locations?

A
  • Subpleural

- Interlobular septal

39
Q

Sign of an early UIP lesion in IPF

A

Cellular fibroblastic foci

40
Q
  • Gradually worsening dyspnea with exertion
  • Dry cough
  • Late development of progressive hypoxemia, cyanosis, and clubbing
  • Smoker, 40-70
  • Microscopic evidence of fibrosis
A

Idiopathic pulmonary fibrosis

41
Q

Median survival after diagnosis of IPF is ____

A

Less than 3 years

42
Q

Systemic non-caseating granulomas with multinucleated giant cells, often in the hilar lymph nodes and/or lung

A

Sarcoidosis

43
Q

How are the microscopic findings in Nonspecific Interstitial Pneumonia (NSIP) different than those in UIP?

A

Develops as EITHER interstitial chronic inflammation OR interstitial fibrosis, NO heterogeneity

44
Q

Typical person to get NSIP

A

Women, 50s, not a smoker, presents w/ dyspnea and cough

45
Q

Genetics in Sarcoidosis (2)

A
  • Family/race clustering

- HLA-A1, HLA-B8

46
Q
  • Cough and dyspnea
  • Patchy subpleural or peribronchial consolidation
  • NO INTERSTITIAL FIBROSIS
  • NO HONEYCOMBING
  • Clusters of fibroblasts/fibrous tissue in terminal airways
  • Normal underlying tissue
A

Cryptogenic organizing pneumonia (COP)

47
Q

Most cases of COP require ___ for treatment

A

Steroid therapy

48
Q

Pneumoconioses - general definition

A

Lung diseases due to chronic responses to inhaled aerosols (mineral dusts, organic dusts, chemical fumes, vapors)

49
Q

Pathogenicity of Pneumoconiosis is determined by what 4 things?

A
  • Particle size (smaller = worse)
  • Particle solubility
  • Level and duration of exposure
  • Intensity of immune response
50
Q

Black pigmented lesions full of dark macrophages within lymphatic tissues

A

Anthracosis - ASYMPTOMATIC coal worker’s pneumoconiosis

51
Q

Macules/nodules of dark macrophages AND collagen networks, located adjacent to respiratory bronchioles

A

Simple Coal Worker’s Pneumoconiosis (CWP)

52
Q

Multiple anthracotic (dark) scars w/ central ischemic necrosis replacing lung parenchyma

A

Progressive massive fibrosis (PMF) - advanced coal workers pneumoconiosis

53
Q

3 complications of PMF

A
  • Respiratory failure
  • Pulmonary HTN
  • Cor pulmonale
54
Q

Anthracosis (black lesions) can also be seen in what 2 populations?

A
  • Smokers

- Urban dwellers

55
Q

Anthracosis can OCCASIONALLY cause what?

A

Centriacinar emphysema

56
Q

Slowly-growing nodular collagen-filled and macrophage-filled scars in the UPPER LUNG or HILAR LYMPH NODES

A

Silicosis

57
Q

Microscopic findings in silicosis nodules/scars

A

Hyalinized (pink) whorls of collagen w/ LITTLE inflammation

58
Q

The slow-growing nodules/scars in Silicosis may do what 3 things over time, depending on circumstances?

A
  • Coalesce into PMF
  • Calcify
  • Get covered by coal dust (often)
59
Q

Birefringent particles within nodular scars - with plane-polarized light

A

Silicosis

60
Q

Patients w/ Silicosis are much more at risk for what 2 things?

A
  • TB

- Lung cancer

61
Q

Fibrous hydrated silicate crystals, known to cause interstitial and pleural fibrosis

A

Asbestos

62
Q

3 things linked to asbestos exposure

A
  • Localized parietal pleural plaques of dense collagen
  • Parenchymal interstitial fibrosis
  • Lung carcinoma / mesothelioma / extra-pulmonary neoplasms
63
Q

2 types of asbestos

Which is worse? Why?

A
  • Amphibole
    • Worse b/c reaches deep lung easier
  • Serpentine
64
Q

Pathogenesis in asbestosis

A
  • Macrophages take up fibers
  • Inflammatory response
  • Interstitial fibrosis via mediators
65
Q

Asbestosis affects which part of lung first? Opposite of what?

A

BASE (opposite of Silicosis)

66
Q

The type of fibrosis in asbestosis is similar to what?

A

UIP (IPF)

67
Q

Asbestosis may progress to what?

A

Honeycomb lung

68
Q

Asbestosis is GREATLY exacerbated by what?

A

Smoking

69
Q

Asbestosis may be complicated by MALIGNANCY of the ___ or ____

A

Lung or Pleura

70
Q

Systemic non-caseating granulomas, often in the hilar lymph nodes and/or lung

A

Sarcoidosis

71
Q

Sarcoidosis is more commonly seen in what populations? (3)

A
  • Young adults (under 40)
  • African americans
  • Women
72
Q

Sarcoidosis pathogenesis

A

Abnormal immune response/regulation to unidentified antigen(s)

73
Q

“Naked” granulomas

A

W/out caseation - seen in Sarcoidosis

74
Q

Common location of Sarcoidosis nodules within the lungs

A

Along lymphatics

75
Q

Other common organs involved in Sarcoidosis besides lungs (5)

A
  • Lymph nodes
  • Liver (hepatomegaly)
  • Spleen (splenomegaly)
  • Bone marrow (phalanges)
  • Skin (nodules, plaque, or macules)
  • Eye (inflammation)
76
Q

Potential symptoms in Sarcoidosis

A
  • Constitutional Sx (weight loss, fever, fatigue, night sweats)
  • Pulmonary (dyspnea, hemoptysis, cough, pain)
77
Q

Most sarcoidosis patients will eventually _____

A

Resolve (w/ or w/o steroid treatment)

78
Q

10% of Sarcoidosis cases develop into ____ (3)

A

Progressive pulmonary fibrosis, PHTN, and cor pulmonale

79
Q

Sarcoidosis is a diagnosis of _____ (explain)

A

Exclusion (rule out TB and fungal infections via cultures/stains)

80
Q

Hypersensitivity pneumonia - general description

A

Immunologic-mediated interstitial inflammation due to inhaled organic antigens (dusts, organisms, etc.)

81
Q

Morphologic findings of hypersensitivity pneumonia

A
  • Interstitial pneumonitis (WBCs)
  • Noncaseating granulomas
  • Interstitial fibrosis
82
Q

Continued antigen exposure in hypersensitivity pneumonia can lead to what?

A

Chronic fibrosis (honeycomb lung)

83
Q

3 main antigens in hypersensitivity pneumonia (and common name of each disease)

A
  • Thermophilic bacteria (air-conditioner lung)
  • Bird excretions/feathers (bird-fancier’s disease)
  • Spore-forming fungi/bacteria (Farmer’s lung)
84
Q

Intra-alveolar brown macrophages, interstitial inflammation, MINIMAL fibrosis

Patient is definitely what?

Treatments?

A

Desquamative Interstitial Pneumonia (DIP)

SMOKER

  • Stop smoking
  • Steroids
85
Q

Common other finding with DIP

A

Emphysema

86
Q

Smoker, fibrosis around the bronchioles, chronic inflammation, brown macrophage clusters in bronchioles, mild fibrosis

A

Respiratory Bronchiolitis-Associated Interstitial Lung Disease

87
Q

Accumulations of dendritic cells (Langerhans cells) into NODULES, w/ fibroblasts, macrophages, and many eosinophils

Patient is typically what?

A

Pulmonary Langerhans cell histiocytosis

Young smoker

88
Q

Potential genetic cause of pulmonary Langerhans cell histiocytosis

A

BRAF mutation

89
Q

Accumulation of surfactant w/in alveolar and bronchiolar spaces

Direct cause?

A

Pulmonary alveolar proteinosis (PAP)

Diminished pulmonary macrophage activity

90
Q

3 causes of PAP

A
  • Autoimmune - GM-CSF autoantibody
  • Congenital
  • Secondary (irritants or immunocompromised)
91
Q
  • Patient presents w/ cough, sputum, and gelatinous material

- Alveoli filled with granular pink precipitate

A

PAP (surfactant accumulation)

92
Q

PAP patients are at risk for what? (2)

A
  • Respiratory failure

- Infection

93
Q

Treatments for PAP (2)

A
  • Whole lung lavage

- GM-CSF