Pulmonary Pathology Part 3 Flashcards

(60 cards)

1
Q

How does idiopathic pulmonary fibrosis damage the lungs and what does it lead to?

A
  • Damage is like waves of inflammatory injury leading to fibrosis
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2
Q

How is idiopathic pulmonary fibrosis diagnosed?

A
  • Classic findings on high-resolution CT scan OR pulmonary biopsy
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3
Q

What will the biopsy in IPF show?

A
  • Usual interstitial pneumonia
  • Normal areas
  • Inflammation
  • FIbroblast foci
  • Peripheral honeycombing
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4
Q

What are some contributing factors to IPF?

A
  • Environmental factors (industrialized societies and smoking)
  • Genetic factors
  • Increasing age (>50)
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5
Q

What does someone with IPF look like clincially?

A
  • Shortness of air
  • Velcro like crackles on exam
  • Restrictive pattern on PFTs
  • Basilar infiltrates (progression to honeycombing)
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6
Q

How long does it take for someone to die from IPF?

A
  • 3-5 years after diagnosis
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7
Q

What are some potential therapies for IPF?

A
  • Lung transplantation

- Medications to arrest fibrosis (tyrosine kinase inhibitors or TGF-B inhibitors)

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8
Q

What is nonspecific interstitial pneumonia (NSIP)?

A
  • Idiopathic
  • Has unique histology
  • Uniform infiltrates and fibrosis
  • No heterogeneity
  • No fibroblast foci
  • No granulomata
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9
Q

What is cryptogenic organizing pneumonia (COP)?

A
  • Formerly BOOP

- Occurs superimposed on prior infection or inflammatory process

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10
Q

What does someone with COP look like?

A
  • Pneumonia-like consolidation

- Presents in fifth/sixth decades

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11
Q

How do you diagnose COP?

A
  • Diagnosis of exclusion

- There isn’t an active infection, not drug or toxin induced, or related to CT disorders

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12
Q

How do you treat COP?

A
  • Usually patients do well on oral steroids
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13
Q

What is the correlation between autoimmune diseases and interstitial lung diseases?

A
  • Autoimmune or CT disorders can manifest as ILD
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14
Q

What happens if ILD occurs as a manifestation of an autoimmune or CT disorder?

A
  • They are not diagnosed as pure diseases

- Prognosis is linked to underlying condition

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15
Q

What is sarcoidosis?

A
  • A systemic manifesting non-caseating granulomata in various organs (90% of cases involve lungs or hilar lymph nodes)
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16
Q

What is the clinical presentation of someone with sarcoidosis?

A
  • Incidental abnormal radiograph
  • Dyspnea
  • <40 years old
  • 10-fold predominance in african americans
  • Elevated serum ACE levels
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17
Q

What are the granuloma inclusions in sarcoidosis?

A
  • Asteroid body (broken down collagen)

- Schaumann bodies

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18
Q

What is the difference between sarcoidosis and hypersensitivity pneumonitis?

A
  • Both have granulomas but hypersensitivity pneumonitis has no fibrosis
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19
Q

What is the cause of hypersensitivity pneumonities?

A
  • Immune reaction to inhaled antigen
  • Pigeon breeder’s lung (protein from bird feces)
  • Farmer’s lung (actinomycetes spores in hay)
  • Hot tub lung (reaction to MAC)
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20
Q

How do you diagnose hypersensitivity pneumonitis?

A
  • History
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21
Q

What is desquamative interstitial pneumonia (DSIP)?

A
  • Restrictive lung presentation
  • Presents in smokers in their 4th or 5th decade
  • Histology will show “stuffed” alveolar spaces full of macrophages
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22
Q

What is the treatment and prognosis for DSIP?

A
  • Stop smoking
  • Corticosteroids
  • Good prognosis (95%)
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23
Q

What is respiratory bronchiolitis interstitial lung disease?

A
  • Part of the spectrum as DSIP but less symptomatic and earlier presentation
  • Often reversible with smoking cessation if caught early
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24
Q

Who presents with RB-ILD?

A
  • Smokers in their 3rd or 4th decade
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25
How do you diagnose RB-ILD?
- Any abnormal radiographs require a prompt biopsy
26
What do we see on histology in RB-ILD?
- Less macrophages - "Peribronchiolar metaplasia" - May have fibrosis in advanced cases
27
What is langerhans cell histiocytosis?
- Stellate lung lesions in young smokers - Progressive scarring leads to cysts - Peripheral cysts may rupture causing pneumothorax
28
What does histology look like with langerhans cell histiocytosis?
- Eosinophils - Langerhan cells - Varying fibrosis and cysts
29
What is pulmonary alveolar proteinosis?
- Impairment of surfactant metabolism due to defect of granulocyte-macrophage colony stimulating factor (GM-CSF)
30
What are the different types of pulmonary alveolar proteinosis?
- Autoimmune - Secondary - Hereditary
31
How is pulmonary alveolar proteinosis treated?
- SubQ GM-CSF
32
What is also seen in pulmonary alveolar proteinosis?
- Copious milky fluid
33
What is pneumoconiosis?
- Reaction by lung to inhaled mineral or organic dust or vapors - Occupational exposure - Air pollution
34
When is pneumoconiosis worse?
- If exposure is repetitive and high - Small particles (able to reach alveoli) - Impaired ciliary clearance (smoking)
35
What is coal workers' pneumoconiosis?
- Disease due to inhaled coal dust, with a spectrum of disease
36
What is the spectrum of disease in coal workers' pneumoconiosis?
- Anthracosis - Coal macule/nodules - Progressive massive fibrosis
37
What is silicosis?
- Disease resulting from inhaled silicon dioxide
38
Where is silicosis seen?
- Seen in mining or quarry work | - Concrete repair or demolition
39
When is the onset of silicosis?
- Insidious onset, can occur after exposure is no longer present
40
What can silicosis progress to?
- May progress to massive pulmonary fibrosis | - Two-fold risk of developing cancer
41
What are the radiologic findings of silicosis?
- Eggshell calcifications (calcified hilar lymph nodes)
42
What is asbestosis?
- Interstitial and pleural disease resulting from inhalation of asbestos fibers
43
What does the risk of asbestosis correlate with? Who typically has asbestosis?
- Risk correlates with asbestos exposure - Insulation workers - Shipyard workers (navy) - Paper mill workers - Oil or chemical refinery workers
44
What are some disease manifestations of asbestosis?
- Pleura: fibrosis/fibrous plaque, effusions, mesothelioma - Lung: interstitial fibrosis, carcinoma - Extrapulmonary neoplasms
45
What are the two types of asbestos fibers? Which one is worse?
- Serpentine | - Amphibole (worst due to straight nature, allowing it to go deeper in lungs)
46
What does asbestos look like on histology?
- Golden dumbbells due to macrophages trying to eat them
47
What does the plaque formation look like in asbestosis?
- Candle Wax drippings on pleura due to hyaline collagen
48
What is mesothelioma?
- Diseases associated with asbestos exposure
49
When does mesothelioma present?
- Decades after exposure | - Lifetime exposure risk is as high as 10%
50
What is a pulmonary infarct?
- Wedge shaped lesion | - Begins as hemorrhagic, then fibrosis sets in
51
What is characteristic of a pulmonary infarct that happens antemortem?
- Lines of Zahn
52
What can obstruct vessels and cause a pulmonary emboli?
- Blood clot - Bone marrow/fat - Cancer - Septic emboli - Air - Foreign material - Parasites
53
What is a talc emboli?
- An emboli seen in IV drug users
54
What else can cause nodules in IV drug users?
- Septic emboli
55
What is a septic emboli?
- Bloodborne infective material | - May occur in endocarditis valve vegetation break off and travels to the lungs
56
What is pulmonary hypertension?
- Pulmonary artery pressure greater than 25 mmHg (have to stick a cath into the heart to obtain)
57
What are some pulmonary hemorrhage syndromes?
- Goodpasture syndrome - Granulomatosis with polyangiitis - Idiopathic pulmonary hemosiderosis
58
What do we see in goodpasture syndrome?
- Immunofluorescence showing a linear pattern of deposition due to anti-basement membrane antibodies
59
What is goodpasture syndrome?
- Antibodies against a non collagenous subunit of collagen IV
60
Where do we see goodpasture syndrome?
- Occurs in males in 2nd or 3rd decade