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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
Multiple anthracotic (dark) scars w/ central ischemic necrosis replacing lung parenchyma
Progressive massive fibrosis (PMF) - advanced coal workers pneumoconiosis
26
3 complications of PMF
- Respiratory failure - Pulmonary HTN - Cor pulmonale
27
Anthracosis can OCCASIONALLY cause what?
Centriacinar emphysema
28
Anthracosis can OCCASIONALLY cause what?
Centriacinar emphysema
29
Interstitial fibrosis leads to what 2 things?
- Low diffusing capacity - Low lung volume - Low lung compliance
30
Honeycomb lung
Advanced stage extensive scarring in any of the restrictive lung disorders
31
Progressive interstitial fibrosis and respiratory failure, unknown cause
Idiopathic pulmonary fibrosis
32
Idiopathic pulmonary fibrosis is THOUGHT to occur secondary to _____
Repeated alveolar epithelial injury w/ pro-fibrotic response
33
Causes of repeated alveolar epithelial injury in idiopathic pulmonary fibrosis
- Tobacco smoke - Fumes, dusts - Viruses - Persistent GI reflux
34
TERT, TERC mutations
Telomerase mutations that make for susceptibility to idiopathic pulmonary fibrosis
35
Molecular cause for the repetitive fibrosis reactions
TGF-beta 1
36
- Patchy interstitial fibrosis | - New fibroblastic foci mixed with more densely fibrotic areas
Usual interstitial pneumonia (UIP) - Morphologic pattern seen in idiopathic pulmonary fibrosis
37
Spaces surrounded by dense collagen, lined with hyperplastic type 2 pneumocytes and bronchial epithelium
Honeycomb fibrosis - common result of restrictive lung diseases
38
In IPF, the fibrosis is usually in which 2 locations?
- Subpleural | - Interlobular septal
39
Sign of an early UIP lesion in IPF
Cellular fibroblastic foci
40
- Gradually worsening dyspnea with exertion - Dry cough - Late development of progressive hypoxemia, cyanosis, and clubbing - Smoker, 40-70 - Microscopic evidence of fibrosis
Idiopathic pulmonary fibrosis
41
Median survival after diagnosis of IPF is ____
Less than 3 years
42
Systemic non-caseating granulomas with multinucleated giant cells, often in the hilar lymph nodes and/or lung
Sarcoidosis
43
How are the microscopic findings in Nonspecific Interstitial Pneumonia (NSIP) different than those in UIP?
Develops as EITHER interstitial chronic inflammation OR interstitial fibrosis, NO heterogeneity
44
Typical person to get NSIP
Women, 50s, not a smoker, presents w/ dyspnea and cough
45
Genetics in Sarcoidosis (2)
- Family/race clustering | - HLA-A1, HLA-B8
46
- Cough and dyspnea - Patchy subpleural or peribronchial consolidation - NO INTERSTITIAL FIBROSIS - NO HONEYCOMBING - Clusters of fibroblasts/fibrous tissue in terminal airways - Normal underlying tissue
Cryptogenic organizing pneumonia (COP)
47
Most cases of COP require ___ for treatment
Steroid therapy
48
Pneumoconioses - general definition
Lung diseases due to chronic responses to inhaled aerosols (mineral dusts, organic dusts, chemical fumes, vapors)
49
Pathogenicity of Pneumoconiosis is determined by what 4 things?
- Particle size (smaller = worse) - Particle solubility - Level and duration of exposure - Intensity of immune response
50
Black pigmented lesions full of dark macrophages within lymphatic tissues
Anthracosis - ASYMPTOMATIC coal worker's pneumoconiosis
51
Macules/nodules of dark macrophages AND collagen networks, located adjacent to respiratory bronchioles
Simple Coal Worker's Pneumoconiosis (CWP)
52
Multiple anthracotic (dark) scars w/ central ischemic necrosis replacing lung parenchyma
Progressive massive fibrosis (PMF) - advanced coal workers pneumoconiosis
53
3 complications of PMF
- Respiratory failure - Pulmonary HTN - Cor pulmonale
54
Anthracosis (black lesions) can also be seen in what 2 populations?
- Smokers | - Urban dwellers
55
Anthracosis can OCCASIONALLY cause what?
Centriacinar emphysema
56
Slowly-growing nodular collagen-filled and macrophage-filled scars in the UPPER LUNG or HILAR LYMPH NODES
Silicosis
57
Microscopic findings in silicosis nodules/scars
Hyalinized (pink) whorls of collagen w/ LITTLE inflammation
58
The slow-growing nodules/scars in Silicosis may do what 3 things over time, depending on circumstances?
- Coalesce into PMF - Calcify - Get covered by coal dust (often)
59
Birefringent particles within nodular scars - with plane-polarized light
Silicosis
60
Patients w/ Silicosis are much more at risk for what 2 things?
- TB | - Lung cancer
61
Fibrous hydrated silicate crystals, known to cause interstitial and pleural fibrosis
Asbestos
62
3 things linked to asbestos exposure
- Localized parietal pleural plaques of dense collagen - Parenchymal interstitial fibrosis - Lung carcinoma / mesothelioma / extra-pulmonary neoplasms
63
2 types of asbestos Which is worse? Why?
- Amphibole - Worse b/c reaches deep lung easier - Serpentine
64
Pathogenesis in asbestosis
- Macrophages take up fibers - Inflammatory response - Interstitial fibrosis via mediators
65
Asbestosis affects which part of lung first? Opposite of what?
BASE (opposite of Silicosis)
66
The type of fibrosis in asbestosis is similar to what?
UIP (IPF)
67
Asbestosis may progress to what?
Honeycomb lung
68
Asbestosis is GREATLY exacerbated by what?
Smoking
69
Asbestosis may be complicated by MALIGNANCY of the ___ or ____
Lung or Pleura
70
Systemic non-caseating granulomas, often in the hilar lymph nodes and/or lung
Sarcoidosis
71
Sarcoidosis is more commonly seen in what populations? (3)
- Young adults (under 40) - African americans - Women
72
Sarcoidosis pathogenesis
Abnormal immune response/regulation to unidentified antigen(s)
73
"Naked" granulomas
W/out caseation - seen in Sarcoidosis
74
Common location of Sarcoidosis nodules within the lungs
Along lymphatics
75
Other common organs involved in Sarcoidosis besides lungs (5)
- Lymph nodes - Liver (hepatomegaly) - Spleen (splenomegaly) - Bone marrow (phalanges) - Skin (nodules, plaque, or macules) - Eye (inflammation)
76
Potential symptoms in Sarcoidosis
- Constitutional Sx (weight loss, fever, fatigue, night sweats) - Pulmonary (dyspnea, hemoptysis, cough, pain)
77
Most sarcoidosis patients will eventually _____
Resolve (w/ or w/o steroid treatment)
78
10% of Sarcoidosis cases develop into ____ (3)
Progressive pulmonary fibrosis, PHTN, and cor pulmonale
79
Sarcoidosis is a diagnosis of _____ (explain)
Exclusion (rule out TB and fungal infections via cultures/stains)
80
Hypersensitivity pneumonia - general description
Immunologic-mediated interstitial inflammation due to inhaled organic antigens (dusts, organisms, etc.)
81
Morphologic findings of hypersensitivity pneumonia
- Interstitial pneumonitis (WBCs) - Noncaseating granulomas - Interstitial fibrosis
82
Continued antigen exposure in hypersensitivity pneumonia can lead to what?
Chronic fibrosis (honeycomb lung)
83
3 main antigens in hypersensitivity pneumonia (and common name of each disease)
- Thermophilic bacteria (air-conditioner lung) - Bird excretions/feathers (bird-fancier's disease) - Spore-forming fungi/bacteria (Farmer's lung)
84
Intra-alveolar brown macrophages, interstitial inflammation, MINIMAL fibrosis Patient is definitely what? Treatments?
Desquamative Interstitial Pneumonia (DIP) SMOKER - Stop smoking - Steroids
85
Common other finding with DIP
Emphysema
86
Smoker, fibrosis around the bronchioles, chronic inflammation, brown macrophage clusters in bronchioles, mild fibrosis
Respiratory Bronchiolitis-Associated Interstitial Lung Disease
87
Accumulations of dendritic cells (Langerhans cells) into NODULES, w/ fibroblasts, macrophages, and many eosinophils Patient is typically what?
Pulmonary Langerhans cell histiocytosis Young smoker
88
Potential genetic cause of pulmonary Langerhans cell histiocytosis
BRAF mutation
89
Accumulation of surfactant w/in alveolar and bronchiolar spaces Direct cause?
Pulmonary alveolar proteinosis (PAP) Diminished pulmonary macrophage activity
90
3 causes of PAP
- Autoimmune - GM-CSF autoantibody - Congenital - Secondary (irritants or immunocompromised)
91
- Patient presents w/ cough, sputum, and gelatinous material | - Alveoli filled with granular pink precipitate
PAP (surfactant accumulation)
92
PAP patients are at risk for what? (2)
- Respiratory failure | - Infection
93
Treatments for PAP (2)
- Whole lung lavage | - GM-CSF

Respiratory 2 (15 decks)

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