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2020 CPR II Pulm Exam 2 > Lung Path 2 - Obstructive (Singh) > Flashcards

Lung Path 2 - Obstructive (Singh) Flashcards

1
Q

What are the four obstructive lung diseases?

A

Emphysema

Chronic bronchitis

Asthma

Bronchiectasis

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2
Q

Which 2 obstructive lung diseases are grouped together and referred to as COPD?

A
  • Chronic bronchitis
  • Emphysema
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3
Q

What are the PFT characteristics of obstructive lung diseases?

A

Characterized by AIR TRAPPING

Decreased flow

Decreased FEV1

LOW FEB1/FVC ration

TLC increased (d/2 inabilty to exhale)

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4
Q

What are the 3 inflammatory changes seen in the chronic bronchitis… or any other small airways of pt with emphysema and even young smokers that narrow the bronchiolar lumen and contribute to obstruction?

A
  • Goblet cell hyperplasia –> mucus plugging of lumen
  • Inflammatory infiltrate in bronchial walls w/ neutrophils, macrophages, B cells, and T cells
  • Thickening of the bronchiolar wall due to smooth m. hypertrophy and peribronchial fibrosis
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5
Q

What are the complications of chronic bronchitis?

A

Bronchiectasis

Squamous metaplasia –> dysplasia –> carcinoma

Death from respiratory infection

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6
Q

Which obstructive lung disease is charactetrized by irreversible enlargement of the airspace distal to the terminal bronchioles, accompanied by destruction of the walls without fibrosis?

A

Emphysema

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7
Q

Of the various types of emphysema, which 2 cause clinically significant airflow obstruction; which is most common?

A
  1. Centriacinar (centrilobular) = most common = Upper lungs
  2. Panacinar (panlobular) = Lower zones
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8
Q

Where are lesions of centriacinar (centrilobular) emphysema most commonly seen and most severe?

A

Upper lobes, in the apical segments

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9
Q

Centriacinar (centrilobular) emphysema occurs predominantly in whom and is often associated with what other lung disorder?

A

Heavy smokers, often in assoc. w/ chronic bronchitis (COPD)

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10
Q

Panacinar (panlobular) emphysema occurs most commonly where in the lungs and is associated with what underlying abnormality?

A
  • Lower zone and anterior margins of lung, usually most severe at bases
  • Associated w/ α1-antitrypsin deficiency

*Image on right*

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11
Q

Which type of emphysema most likely underlies many cases of spontaneous pneumothorax in young adults?

A

Distal acinar (paraseptal) emphysema

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12
Q

What are some of the inflammatory mediators released by macrophages and resident epithelial cells which influence the development of emphysema?

A
  • Leukotriene B4
  • IL-8
  • TNF
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13
Q

The pathogenesis of emphysema is related to an imbalance between which factors?

A

Neutrophil elastase (protease) and anti-proteases (α<strong>1</strong>-antitrypsin)

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14
Q

Which gene related to protection from oxidatie stress may be mutated in emphysema and other smoking-related lung diseases?

A

NRF2

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15
Q

α1-antitrypsin is encoded by which locus and on what chromosome?

A

Proteinase inhibitor (Pi) on chromosome 14

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16
Q

Loss of elastic tissue in the walls of alveoli in emphysema causes respiratory bronchioles to do what during expiration?

A

Collapse —> functional airflow obstruction

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17
Q

What is the characterisitc gross morphology seen in advanced emphysema?

A
  • Enlarged lungs which often overlap the heart
  • Large alveoli seen on cut surface of fixed lungs
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18
Q

Microscopically, in emphysema, the large alveoli are separated by what and have fibrosis where?

A

Thin septa w/ only focal centriacinar fibrosis

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19
Q

Which sx of emphysema typically appears first and what are some other associated sx’s that may be present?

A
  • Dyspnea that’s progressive
  • Cough or wheezing may be present, easily confused w/ asthma
  • Weight loss is common; may be severe enough as to suggest cancer
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20
Q

What value and test is the key to diagnosis of emphysema?

A

Impaired expiratory airflow, best measured w/ spirometry

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21
Q

What develops in association with secondary HTN, which is also an indicator of poor prognosis in pt w/ emphysema?

A

Cor pulmonale and eventual CHF, related to 2’ pulmonary HTN

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22
Q

How will a CXR of predominant bronchitis differ from predominant emphysema?

A
  • Bronchitis = prominent vessels; large heart
  • Emphysema = hyperinflation; small heart
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23
Q

Obstructive overinflation is commonly caused by what; why is the form of emphysema significant?

A
  • Tumor or foreign object
  • Can be life-threatening emergency, due to affected portion distending and compressinf remaining lung
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24
Q

What is a complication which may arise w/ Bullous Emphysema?

A

Rupture of bullae –> pneumothorax

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25
Q

What is the primary or initiating factor in the pathogenesis of chronic bronchitis?

A

Exposure to noxious or irritating inhaled substances such as tobacco smoke and dust from grain, cotton, and silica

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26
Q

What is the earliest feature seen in the pathogenesis of Chronic Bronchitis; over time there is a marked increase in what cell type?

A
  • Mucus hypersecretion
  • Assoc. w/ hypertrophy of the submucosal glands in trachea and bronchi
  • With time there is marked ↑ in goblet cells in small airways
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27
Q

What is the role of infection in Chronic Bronchitis?

A
  • Significant in maintaining the pathologic state
  • Critical in producing acute exacerbation
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28
Q

What are the characteristic gross morphological features of chronic bronchitis; enlargement of what?

A
  • Mild inflammation of airways (predominantly lymphocyte infiltrates)
  • Enlargement of the mucus-secreting glands of the trachea and bronchi
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29
Q

Although the number of goblet cells increase slightly in Chronic Bronchitis, what is the major change seen?

A

mucous gland hyperplasia –> leads to epithelum damage in the airways

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30
Q

The increase in size of the mucous glands in chronic bronchitis can be assessed via what ratio?

A

Ratio of thickness of mucous gland layer to the thickness of the wall btw the epithelium and cartilage (Reid index)

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31
Q

What morphological changes may the epithelium exhibit in Chronic Bronchitis?

Why is it concerning?

A

Squamous metaplasia (primed for proliferation and growth)

–> dysplasia –> carcinoma

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32
Q

In the most severe cases of Chronic Bronchitis, there may be obliteration of the lumen due to fibrosis and this is known as?

A

Bronchiolitis obliterans

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33
Q

Long-standing severe chronic bronchitis commonly leads to what (cause of death)?

A

Cor pulmonale (RVF) –> HF

34
Q

Asthma is distinguished from chronic bronchitis and emphysema by the presence of what feature?

A

Reversible bronchospasm

35
Q

Early-onset allergic asthma is associated with inflammation due to what type of T cells and has good response to what tx?

A
  • TH2 helper T cell inflammation
  • Responds well to corticosteroids
36
Q

Respiratory infections due to what are common triggers of non-atopic asthma and may act in synergy with enviornmental allergens to cause atopic asthma?

A

Viruses (i.e., rhinovirus, parainfluenza, and respiratory syncytial virus)

37
Q

Non-atopic asthma attacks may be triggered by seemingly innocuous events, such as what?

A
  • Exposure to cold
  • Exercise
38
Q

Aspirin-sensitive asthma occurs most commonly in pt’s with what underlying disorders?

A

Recurrent rhinitis and nasal polyps

39
Q

Pt’s with aspirin-sensitive asthma suffer from typical asthma attack sx’s during an attack…

What is different about their clinical presenation?

A
  • Asthmatic attacks​ and Urticaria (aka hives) during an attach

Samter’s Triad

  • ASA sensitivity
  • nasoplyps
  • recurrent rhinositis
40
Q

Describe how aspirin inhibiting cyclooxygenase plays a role in the pathogenesis of aspirin-sensitive asthma?

A

Leads to rapid ↓ in PGE2, which normally inhibit leukotrienes B4, C4, D4, and E4

41
Q

As asthma progresses and becomes more severe, there is ↑ local secretion of growth factors, which induce what 5 changes?

A
  • Mucus gland hypertrophy
  • Smooth m. proliferation
  • Angiogenesis
  • Fibrosis
  • Nerve proliferation
42
Q

A fundamental abnormality in the pathogenesis of asthma is an exaggerated response by which immune cells to normally harmless enviornmental allergens?

A

TH2 response

43
Q

What is the role of IL-4, IL-5, and IL-13 released from TH2 cells in asthmatic patients?

A
  • IL-4 stimulates class switching to IgE
  • IL-5 activates eosinophils
  • IL-13 stimulates mucus secretion and IgE production by B cells
44
Q

Other than TH2 cells what other type of T cell is seen in the late-phase reaction of asthma and what is it’s function?

A

TH17 produce IL-17 –> recruit neutrophils

45
Q

The bronchoconstriction characteristic of the early phase (immediate hypersensitivity) of asthma is triggered by what?

A

Direct stimulation of Vagal (parasympathetic)receptors by reflexes triggered via mediators produced bymast cells and other immune cells

46
Q

Which 2 types of mediators play a clear role in the bronchospasm, increased vascular permeability, and airway smooth muscle constriction seen in asthma?

A
  1. Leukotrienes C4, D4, E4
  2. ACh released from intrapulmonary parasympathetic nerves
47
Q

Which factors released from eosinophils in the late phase reaction of asthma cause damage to the epithelium?

A
  • Major basic protein
  • Esoinophil cationic protein
48
Q

Leukotrienes C4, D4, E4 are responsible for what 3 pathogenic processes in asthma?

A
  • Bronchoconstriction
  • Mucus secretion
  • ↑ vascular permeability
49
Q

There is an increased incidence of what 2 other allergic disorders in those with atopic asthma?

A
  • Allergic rhinitis
  • Eczema
50
Q

One susceptibility locus for asthma is located on what chromosome; near the gene cluster encoding what cytokines?

A
  • Chromosome 5q
  • IL-3, IL-4, IL-5, IL-9 and IL-13 + IL-4 receptor
51
Q

Polymorphisms in which interleukin gene have the strongest and most consistent associations w/ asthma or allergic disease?

A

IL13 gene

52
Q

Polymorphisms in which gene encoding a metalloproteinase, may be linked to ↑ proliferation of bronchial smooth m. cells and fibroblasts –> bronchial hyperreactivity and subepithelial fibrosis?

A

ADAM33

53
Q

Variants of which interleukin receptor gene is associated w/ atopy, elevated total serum IgE and asthma?

A

IL-4 receptor gene variants

54
Q

Increased serum levels and lung expression of which chitinase-like glycoprotein is correlated w/ disease severity, airway remodeling, and decreased pulmonary function in asthmatics?

A

YKL-40

55
Q

What is the most striking gross finding in pt’s dying of acute severe asthma (status asthmaticus)?

A

Occlusion of bronchi and bronchioles by thick, tenacious mucus plugs, which often contain shed epithelium

56
Q

The idea that microbial exposure during early development reduces the later incidence of allergic (and some autoimmune) diseases has been known as what?

A

Hygiene hypothesis

57
Q

What are 2 characteristic findings in the sputum or bronchoalveolar lavage specimens in a pt w/ severe asthma?

A
  • Curschmann spirals = Coiled mucus plus
  • Numerous eosinophils and Charcot-Leyden crystals composed of an eosinophil protein called galectin-10
58
Q

The characteristic histologic finding of “airway remodeling” in pt w/ asthma includes what 5 major changes?

A
  • Thickening of airway wall
  • Sub-basement membrane FIBROSIS
  • ↑ vascularity
  • ↑ in size of submucosal glands and # of goblet cells
  • Hypertrophy and hyperplasia of the bronchial wall muscle
59
Q

What are the 4 contributors to chronic irreversible airway obstruction in asthma?

A
  • Muscular bronchoconstriction
  • Acute edema
  • Mucus plugging
  • Airway remodeling
60
Q

What are the cardinal sx’s of asthma?

A
  • Chest tightness
  • Dyspnea
  • Wheezing
  • Cough (with or w/o sputum production)
61
Q

Chronic irreversible airway obstruction will show a decreased response to what?

A

Therapeutic agents –> Bronchodilators and/or Corticosteroids

62
Q

What are the 2 major conditions associated with Bronchiectasis and are often both necessary for its development?

A
  • Obstruction
  • Infection
63
Q

Disorder in which destruction of smooth muscle and elastic tissue by chronic necrotizing infections leads to permanent dilation of bronchi and bronchioles

A

Bronchiectasis

64
Q

List congenital or hereditary conditions which may lead to Bronchiectasis?

A
  • Cystic Fibrosis
  • Intralobar sequestration
  • Primary ciliary dyskinesia
  • Kartagener syndromes
65
Q

What are some acquired conditions that may lead to necrotizing inflammatory response (bronchiectasis)?

A

Allergic Bronchopulmonary Aspergillosis (ABPA)

and Tuberculosis (chronic infection)

66
Q

Many cases of Bronchiectasis lack any association with another disease process and therefore are what?

A

Idiopathic

67
Q

Which 3 organisms are the most common causes of lung infection in pt with CF?

A
  • Staphylococcus aureus
  • Haemophilus influenzae
  • Pseudomonas aeruginosa
68
Q

Which autoimmune disorders and other conditions are associated w/ developing Bronchiectasis?

A
  • Rheumatoid Arthritis
  • SLE
  • IBD
  • COPD
  • Post-transplantation
69
Q

In CF the primary defect in ion transport leads to defective what in the lungs?

A
  • Mucociliary action + airway obstruction by thick viscous secretions
  • Sets stage for chronic bacterial infections
70
Q

Primary ciliary dyskinesia is due to mutations in what?

A

Ciliary motor proteins (i.e., Dynein arm of microtubules)

71
Q

Half of the pt’s with primary ciliary dyskinesia have what syndrome and what is the triad of this syndrome?

A
  • Kartagener syndrome
  • Marked by situs inversus + bronchiectasis + sinusitis
72
Q

Males with Kartagener Syndrome tend to be what?

A

Infertile, as result of sperm dysmotility

73
Q

Allergic bronchopulmonary aspergillosis occurs in what 2 underlying conditions?

A
  • Asthma
  • Cystic Fibrosis
74
Q

Which stain can be used to demonstrate Aspergillus and what is seen?

A
  • Silver stain
  • Aggregates of fungal hyphae
75
Q

What are characteristic findings in the seurm of someone with Allergic Bronchopulmonary Aspergillosis?

A

High serum IgE and serum Abs to Aspergillus

76
Q

Bronchiectasis usually affects which lobes of the lung, particularly which air passages, and is most severe where?

A
  • Lower lobes bilaterally
  • Particularly air passages that are vertical
  • Most severe in the more distal bronchi and bronchioles
77
Q

Which lung disease will have dilated airways, sometimes up to 4x normal size?

A

Bronchiectasis

78
Q

What are the signs/sx’s of Bronchiectasis?

A
  • Severe, persistent cough w/ foul smelling, sometimes bloody sputum
  • Dyspnea and orthopnea in severe cases
  • Occasionaly hemoptysis, which can be massive!
79
Q

What are 3 potential complications of Bronchiectasis?

A
  • Cor pulmonale
  • Brain abscess
  • Amyloidosis
80
Q

How do you diagnoze Chronic Bronchitis?

A

Persistent cough with sputum production for 3months out of 2 consecutive years.

Pathophysiologic findings will include mucus gland hyperplasia with damage to airway epithelium

2020 CPR II Pulm Exam 2 (28 decks)

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