Pulm Path Pt 2 Flashcards

1
Q

What does idiopathic pulmonary fibrosis lead to?

A

IPF damages pulmonary tissue with waves of inflammatory injury leading to fibrosis

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2
Q

What presents as:

  • basilar infiltrates -> “honeycomb lung”
  • dyspnea
  • crackles on auscultation
A

IPF

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3
Q

What are the contributing factors of IPF?

A
  • environmental: industrialized societies, smoking
  • genetic factors
  • increasing age (>50)
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4
Q

What is expected on pulmonary biopsy of IPF?

A
  • normal areas
  • inflammation
  • fibroblast foci
  • peripheral honeycombing
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5
Q

What are the potential therapies of IPF?

A
    • lung transplantation

- medication to arrest fibrosis: tyrosine kinase inhibitors, TGFb inhibitors

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6
Q

Why does non-specific interstitial pneumonia (NSIP) have a much better prognosis than IPF?

A

It is more diffuse

  • NOT non-specific
  • uniform infiltrates and fibrosis
  • no heterogeneity
  • no fibroblast foci
  • no granulomata
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7
Q

50-60 year old patient presents with pneumonia-like consolidation
- fibroblast foci, aka Masson bodies (organizing plugs of connective tissue that look like cotton candy)

A

Cryptogenic Organizing Pneumonia (COP)

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8
Q

What can be given to control COP?

A

excellent prognosis (full recovery) with oral steroid**

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9
Q

Why is COP a diagnosis of exclusion?

A

it is not infection, drug or toxin-induced, or related to connective tissue disorders

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10
Q

What are the three autoimmune/connective tissue diseases that can manifest as Interstitial Lung Disease (ILD)?

A
  1. Rheumatoid arthitis
  2. Systemic sclerosis
  3. Lupus erythematosus
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11
Q

What is prognosis linked to in ILD?

A

treatment of the underlying condition

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12
Q

What does sarcoidosis manifest as?

A

** non-caseating (non-necrotizing)** granulomata

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13
Q

African American pt, less than 40 years old, with incidental abnormal radiograph or dyspnea
- elevated serum ACE levels

A

sarcoidosis

  • likely immune-related etiology, possible genetic predisposition
  • death may occur from pulmonary, cardiac, or neurologic involvement

BERNIE MAC

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14
Q

What are the 5 stages of sarcoidosis on the basis of chest radiographs?

A
Stage 0: no abnormalities
Stage 1: lymphadenopathy
Stage 2: lymphadenopathy + pulmonary infiltration
Stage 3: pulmonary infiltration
Stage 4: fibrosis

NOTE: stages do not always occur in order, 20% of patients have progressive lung disease

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15
Q

If you see an Asteroid body or Schaumann bodies on a histo slide, what should you always think of?

A

sarcoidosis

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16
Q

What is hypersensitivity pneumonitis?

A

immune reaction to inhaled antigen

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17
Q

Pigeon-breeder’s lung, farmer’s lung and hot tub ling are all examples of what?

A

hypersensitivity pneumonitis

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18
Q

What does the diagnosis of hypersensitivity pneumonitis require?

A

thorough history!!!!

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19
Q

When would you see an AIRWAY-centered granulomata with associated lymphocytes?

A

hypersensitivity pneumonitis

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20
Q

40-50 year old patient, smoker, presents with restrictive lung disease
- histology shows very characteristic “stuffed” alveolar spaces full of macrophages

A

Desquamative Interstitial Pneumonia (DSIP)

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21
Q

What is the treatment for DSIP?

A
  • stop smoking!
  • corticosteriods
  • good prognosis: >95% survival at 5 years
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22
Q

Within the spectrum of DSIP, but less symptomatic and earlier presentation (30-40 years old)
- smoking-related (dose dependent)

A

Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD)

NOTE: often reversible with smoking cessation if caught early

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23
Q

What is required to diagnose RB-ILD?

A

radiographic abnormalities on chest CT prompt biopsy

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24
Q

What is seen histologically in RB-ILD?

A
  1. macrophages present to a lesser extent
  2. “peribronchiolar” metaplasia
  3. may have fibrosis in advanced cases
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25
Q

Young smoker, presents with stellate lung lesion

A

Langerhands Cell Histiocytosis (LCH)

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26
Q

What is seen histologically in LCH?

A
  • eosinophils
  • langerhands cells (immature dendritic cells)
  • varying fibrosis and cysts
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27
Q

Why would a patient with LCH present with pneumothorax?

A
  1. progressive scarring leads to cysts

2. peripheral cysts may rupture!!

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28
Q

Can LCH be reversed with smoking cessation?

A

yes!

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29
Q

What lab values are considered a giveaway for LCH?

A

CD1a positive, and abundant eosinophils

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30
Q

Impairment of surfactant metabolism due to defect of granulocyte-macrophage color stimulating factor (GM-CSF)
- accumulation of surfactant proteins throughout alveoli and airspaces

A

Pulmonary alveolar proteinosis

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31
Q

What are the typical demographics of pulmonary alveolar proteinosis patient?

A
  • autoimmune pt (middle-aged female most likely)

- fluid collected from bronchioalveolar lavage shows copious amount of milky fluid

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32
Q

How can you treat pulmonary alveolar proteinosis?

A

GM-CSF (subcutaneous)

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33
Q

What do pulmonary infarcts look like?

A

wedge-shaped lesions

- begin as hemorrhagic, then fibrosis sets in

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34
Q

What is a saddle pulmonary embolism?

A

an emboli that wedges in primary arterial trunk

  • patient dies of acute cor pulmonale because right heart can pump against pressure buildup
  • they die before infarction/necrosis sets in
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35
Q

What is a hallmark of antemortem (before death) clots?

A

Lines of Zahn

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36
Q

What can obstruct vessels in a pulmonary emboli?

A
  • blood clot
  • bone marrow/fat
  • cancer (tumor embolus)
  • septic emboli
  • air
  • foreign material
  • parasites (dorofilaria)
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37
Q

When would you see a bone marrow embolism?

A

after trauma

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38
Q

What is a talc embolism (looks shiney on gross biopsy) characteristic of?

A

IV drug users

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39
Q

What can cause nodules in drug users?

A

**septic emboli **

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40
Q

What is a septic emboli?

A

bloodborne infective material, valve vegetations break off and manifest in other sites -> LUNGS!

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41
Q

When might you see a septic emboli?

A

endocarditis

NOTE: look for Janeway lesions on skin, Roth spots in retina, and splinter hemorrhages in nailbed

42
Q

What is the definition of pulmonary HTN?

A

pulmonary artery pressure (PAP) greater than 25 mmHg

43
Q

What are the subgroups of pulmonary HTN?

A
  1. pulmonary arterial HTN (PAH)
  2. secondary to left heart failure
  3. secondary to chronic pulmonary parenchymal disease
  4. secondary to thromboembolic pulmonary disease
  5. multifactorial
44
Q

What histological finding is very characteristic of pulmonary HTN?

A

plexiform lesion

45
Q

What is medial hypertrophy?

A

when there is smooth muscle in small arteries

- is a good indicator of pulmonary HTN

46
Q

What do Goodpasture syndrome, granulomatosis with polyangiitis, and idiopathic pulmonary hemosiderosis have in common?

A

they are all pulmonary hemorrhage syndromes

47
Q

What is a hallmark of pulmonary hemorrhage syndrome on a histo slide?

A

** hemosiderin-laden macrophages **

48
Q

What are immunofluorescence showing a LINEAR pattern of deposition due to anti-basement membrane antibodies characteristic of?

A

Goodpasture syndrome

- can see RBC in lungs (not good!)

49
Q

What does Goodpasture syndrome result from?

A

antibody-mediated damage to basement membranes in the lung and kidney
- Ab against collagen :(

50
Q

What type of hypersensitivity reaction is Goodpasture syndrome?

A

type 2

51
Q

What does granulomatosis with polyangiitis remind you of on a histo slide?

A

Tb (or other serious infection)

52
Q

What is the most common cause of sepsis and septic shock?

A

pneumonia

53
Q

What are the two categories of pneumonia?

A
  1. clinical setting

2. anatomic distribution

54
Q

What are the two types of “anatomic distribution” pneumonia?

A
  1. bronchopneumonia

2. lobar pneumonia

55
Q

What are the 4 stages of lobar pneumonia?

A
  1. congestion (vascular engorgement)
  2. red hepatization (red cells + inflamm)
  3. grey hepatization (inflamm + debris)
  4. resolution (fibrosis, macrophage clean-up)
56
Q

What is considered a system-specific process of acute inflammation?

A

lobar pneumonia

57
Q

What are the complications of lobar pneumonia?

A

solidification in the lung due to intense inflammatory process

  • abscess
  • empyema (puss-filled)
  • bacteremia can spread! very dangerous!
58
Q

What organisms can lead to community-acquired bacterial pneumonia (CAP)?

A
  • S. pneumoniae
  • H. influenzae
  • S. aureus
  • K. pneumoniae
  • P. aeruginosa
  • L. pneumophilia
  • M. pneumoniae
59
Q

What is the most common cause of CAP?

A

S. pneumoniae

- lancet-shaped gram positive diplococci (pairs and chains)

60
Q

What population is vaccination for S. pneumoniae recommended for?

A

infants, patients older than 65 years, and respiratory disease/smoking population

61
Q

What organism causes virulent pneumonia in children?

A

H. influenzae

- recommended vaccination for type B for children younger than 5 years

62
Q

What organism causes abscess formation

A

S. aureus

- seen in IV drug users

63
Q

What organism causes currant jelly sputum

A
Klebsiella pneumoniae (gram negative)
- seen in alcoholics
64
Q

What population would you see Pseudomonas aeruginosa infection?

A

cystic fibrosis

NOTE: aeruginosa = copper rust

65
Q

Which type of pneumonia presents as:

  • abrupt onset
  • predominant respiratory symptoms
  • consolidation on CXR
  • older adults or young children
A

Typical pneumonia

66
Q

Which type of pneumonia presents as:

  • slower onset
  • systemic symptoms
  • patchy infiltrates on CXR
  • young adults/teens/older children
A

“Atypical/walking” pneumonia

67
Q

What do “patchy infiltrates” look like on CXR?

A

very fine white lines

68
Q

What does typical pneumonia look like on CXR?

A

very dark/consolidated areas

69
Q

What organisms cause typical pneumonia?

A
  • S. pneumoniae
  • H. influenzae
  • S. aureus
  • K. pneumoniae
  • P. aeruginosa
70
Q

What organisms cause “atypical” pneumonia?

A
  • Mycoplasma pneumoniae has no cell wall!
  • Legionella pneumophila
  • chlamydia pneumonia
  • chlamydia psittaci
71
Q

What is the smallest free-living, self-replicating microorganism that lacks a cell wall?

A

Mycoplasma pneumoniae

- is tiny! smaller than cilia, so can progress very far down respiratory tract

72
Q

Gram negative bacillus, grows in warm freshwater (AC units/misters/hot tubs)
- airborne disease (not person-person spread)

A

Legionella pneumophila

73
Q

Influenza, SARS and RSV are examples of what?

A

community-acquired viral pneumonia

74
Q

What are the 2 proteins that classify influenza virus?

A
  1. hemagglutinin (attaches to cells)

2. neuraminidase (allows release of replicated virus from cells)

75
Q

What is the MOA of neuraminidase?

A

it is a viral enzyme that cleaves the hemagglutinin tether anchoring the viron to the infected body cell
- once the tether is cleaved, the virion is released to infect other body cells

76
Q

What does Tamiflu do?

A

it inhibits neuraminidase, so virion particles get stuck, and cannot infect other cells

77
Q

Describe antigenic DRIFT

A
  • epidemic
  • minor changes to proteins (antigens) on the virus, allow increased speed
  • similar enough to original virus to allow for some immunity
78
Q

Describe antigenic SHIFT

A
  • pandemic
  • genomic alterations with major resulting changes to protein structure
  • naive immunity for almost all people
  • picked up from animal gene products
79
Q

What was so bad about H1N1 virus?

A

it was a recombination of bird and swine virus

- spread so fast because we had no immunity to its recombination

80
Q

What disease is caused by coronavirus, originating in China, with a worldwide outbreak from 2002-2004?

A

Severe Acute Respiratory Syndrome (SARS)

81
Q

What is the most likely cause of pneumonia in neonates/

A

bacterial: Group B strep, gram negative bacilli, Listeria

82
Q

What is the most likely VIRAL cause of pneumonia in children older than 1 month?

A
  • respiratory syncytial virus

- parainfluenza virus, influenza A/B adenovirus, rhinovirus

83
Q

What is the most likely BACTERIAL cause of pneumonia in children older than 1 month?

A

S. pneumoniae, H. influenzae, M. catarrhalis, S. aureus

84
Q

What are additional bacterial considerations for pneumonia in older children/adolescents?

A

M. pneumoniae, C. pneumoniae

85
Q

Patient presents with symptoms of rhinorrhea, cough

  • wheezing** and dyspnea
  • tachypnea
  • cyanosis
A

Respiratory Syncytial virus (RSV)
- virus replication results in epithelial-cell sloughing, inflammatory cell infiltration, edema, increased mucus secretion, and impaired ciliary action

86
Q

What is the general presentation of bacterial pneumonia?

A
  • abrupt onset
  • not associated with epidemics
  • may have associated bacteremia
  • high grade fever
  • crackles on lung exam
  • lobar or consolidated appearance
  • may involve pleura
  • responds quickly to appropriate antibiotics
87
Q

What is the general presentation of viral pneumonia?

A
  • gradual onset
  • epidemics are common
  • not typically associated with viremia
  • no fever/low grade fever
  • wheezes on lung exam
  • diffuse infiltrates on CXR
  • doesn’t typically involve pleura
  • will not respond to antibiotics
88
Q

Lung abscesses are complications of pneumonia caused by which two organisms?

A
  • S. aureus

- Klebsiella pneumoniae

89
Q

When are you likely to see a lung abscess due to aspiration?

A
  • chronic alcoholics
  • elderly patients, stroke
  • anaerobic bacteria
90
Q

What is aspiration pneumonia?

A
  • abscess formation in acute bacterial pneumonia
  • food particle in inflammatory exudate indicating aspiration
  • large neutrophilic exudate causing inflammation
91
Q

When are you most likely to see a caseating granulomata?

A

Tb

92
Q

What is a Ghon complex?

A

area of hyaline/consolidated necrosis with a distinct border, giant cells on the outside of the border

93
Q

What is chronic pneumonia?

A

pneumonic process lasting for months in an immunocompetent patient

94
Q

What 3 fungi can cause chronic pneumonia?

A
  1. Histoplasma
  2. Blastomycosis
  3. Coccidiomycosis
95
Q

Which fungi:

  • is endemic in midwest and Carribean (Ohio/Mississippi river valleys)
  • typically presents as subclinical infection with granulomatous response (calcifications/coin lesions on CXR)
  • characteristic yeast form shows pumpkin seed morphology
  • runs aggressive course, especially in immunocompromised patients
A

Histoplasma capsulatum

96
Q

Which fungi:

  • is endemic in central and SE US (Ohio/MI river valleys)
  • in the lungs, infection yields granulomatous response
  • characteristic yeast forms show broad-based budding
  • also infects skin and rarely, spreading infection
A

Blastomyces dermatitides

97
Q

Which fungi:

  • is endemic in SW US and Mexico
  • in the lungs, infection yield a granulomatous response with eosinophils
  • often a subclinical, self-limited disease
  • can produce spreading infection, especially in immunocompromised patients
A

Coccidiodes immitis

Histo slides will show large sphere filled with endospores*

98
Q

Which fungi:

  • is opportunistic
  • causes an AIDS-defining illness
  • has characteristic cup-shaped yeast forms
  • can be diffuse or focal cysts on radiograph
A

Pneumocystic jiroveci (carinii)

99
Q

Immunocomprimised (or elderly) patient presents with thin mycobacteria seen on slender red forms on acid-fast stainging

A

Mycobacterium avium complex (MAC)

100
Q

Post lung transplantation, why are regular biopsies necessary?

A

they are needed to discriminate infection from rejection (which can also produce infiltrates and fever)

NOTE: rejection shows mononuclear infiltrates around vessels