Singh Pathology Respiratory Pathology #3 Flashcards

1
Q

What is the most common type of interstitial lung disease processes?

A
  • Idiopathic pulmonary fibrosis
  • This damages pulmonary tissue with waves of inflammatory injury leading to fibrosis
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2
Q

What does Usual Interstitial Pneumonia consist of and what is it?

A
  • Pathological diagnosis of IPF
  • Consists of normal areas, inflammation, fibroblast foci and peripheral honeycombing
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3
Q

What factors contribute to getting IPF?

A
  • Environmental such as industrialized societies and smoking
  • Genetics
  • Increasing age
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4
Q

Clinical findings in Idiopathic pulmonary fibrosis?

A
  • Dyspnea
  • Crackles (velcro like)
  • Restrictive PFT
  • Basilar infiltrates progressing to honeycomb pattern
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5
Q

How can you differentiate emphysema honeycombing vs IPF?

A

thick areas between fibrosis on histology and grossly differentiate IPF from emphysema

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6
Q

Prognosis of IPF? Therapy?

A
  • Progressive- most patients die 3-5 yrs
  • Therapies include
    • lung transplant
    • Tyrosine kinase inhibitors
    • TGF-B inhibitors
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7
Q

What is Non specific Interstitial Pneumonia? What makes up the histology?

A
  • Idiopathic pneumonia
  • Unique histology
    • Uniform infiltrates and fibrosis
    • NO heterogeneity
    • NO fibroblast foci
    • NO granulomata
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8
Q

Describe Cryptogenic Organizing Pneumonia.

A
  • Superimposed on prior infection or inflammatory process
  • Presents with a pneumonia like consolidation in 50-60s
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9
Q

Why is COP a diagnosis of exclusion?

A
  • Not an active infection, drug or toxin induced or related to CTD
  • If you give steroids it clears up!
  • Good prognosis with full recovery
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10
Q

If a patient has RA, systemic sclerosis, or SLE and develops IPF, NSIP, or COP how do you diagnose these patients?

A
  • Connective Tissue Disease ILD
    • prognosis is based on the CTD the person has
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11
Q

What two diseases have a prominent granulomatous pattern?

A
  • Sarcoidosis
  • Hypersensitivity Pneumonia
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12
Q

What is sarcoidosis? How does it present?

A
  • Non caseating granulomata in various organs
  • mostly in lungs or hilar lymph nodes
  • Presents incidentally on radiopgraph or with dyspnea
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13
Q

What is this?

A

Schaumann bodies associated with Sarcoidosis

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14
Q

What is this?

A

Asteroid body associated with sarcoidosis

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15
Q

Clinical presentation of patients with Sarcoidosis?

A
  • <40 yr old
  • 10x higher in African Americans
  • Elevated ACE levels
  • Likely immune related etiology possible genetic presdisposition
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16
Q

What do patients die from with sarcoidosis?

A
  • Pulmonary
  • Cardiac
  • Neurologic
17
Q

What is Hypersensitivity Pneumonitis?

A
  • Immune reaction to inhaled antigen
    • pigeon breeders lung
    • Farmers lung
    • Hot tub lung
  • Airway centered granulomata with associated lymphocytes
18
Q

How do you diagnose Hypersensitivity Pneumonitis?

A
  • Very detailed history
19
Q

Why is IPF/UIP a poor prognosis?

A

The patients lungs become fibrotic

20
Q

Why is COP a good prognosis?

A

Loose wisps of early fibroblast foci are poorly established so they easily melt away with steroids

21
Q

Why are the granulomas in hypersensitivity pneumonia so ill defined and mixed with other inflammatory cells?

A

All of the inflammation ins a reaction to an inhaled substance

22
Q

What is Desquamative Interstitial Pneumonia?

A
  • Smokers in 4-5th decades
  • Restrictive lung disease presentation
  • Histology shows stuffed alveolar spaces of macrophages
23
Q

Prognosis & treatment of DSIP?

A
  • Good prognosis
  • Treatment is to stop smoking and use corticosteroids
24
Q

What is Respiratory Bronchiolitis Interstitial Lung Disease?

A
  • Part of a spectrum with DSIP but less symptomatic and earlier seen in the 3rd-4th decades
25
Q

How do you diagnose RB-ILD?

A
  • CT scan
  • Then biopsy which shows
    • macrophages to lesser extent
    • Peribronchiolar metaplasia
    • fibrosis in adv cases
26
Q

Langerhans Cell Histiocytosis, who gets it, what does histology show?

A
  • Young smokers present with stellate lung lesions
  • Progressive scarring leads to cysts
  • Peripheral cysts may rupture presenting with pneumothorax
  • Histology shows
    • eosinophils
    • Langerhans cells
    • Fibrosis and cysts
27
Q

What is pulmonary alveolar proteinosis? Tx?

A
  • Impairment of surfactant metabolism due to defect of GM-CSF
  • Leads to accumulation of surfactant proteins throughout alveoli and airspaces
  • Tx with GM-CSF
28
Q

What is happening?

A

Pulmonary alveolar proteinosis

occurs in setting of autoimmune disease 90% of time

29
Q

What is Pneumoconiosis?

A
  • Reaction by lungs to inhaled mineral or organic dust or vapors
    • important to identify occupational exposures and air pollutions
30
Q

What makes pneumoconoisis worse?

A
  • Exposure is high and repetitive
  • Size of particles is small (1-5 um allows particle to reach alveoli)
  • Impaired ciliary clearance
31
Q

Describe the spectrum of disease seen with coal workers pneumoconiosis?

A
  • Anthracosis
  • Coal macules/nodules
  • Progressive massive fibrosis
  • most people will not have progressive disease
32
Q

What is Silicosis? Who gets it?

A
  • Disease resulting from inhaled silicon dioxide
    • seen in those who did mining/quarry work
    • Concrete repair/demo
33
Q

___ may progress to massive pulmonary fibrosis and has a 2 fold risk of developing cancer as opposed to CWP.

A

Silicosis may progress to massive pulmonary fibrosis and has a 2 fold risk of developing cancer as opposed to CWP.

34
Q

What is the most deadly of the pneumoconiosis?

A

Asbestosis

35
Q

Who is at risk for asbestosis?

A
  • Insulation workers
  • Shipyard workers (navy)
  • Paper mill workers
  • Oil/chemical refinery workers
36
Q

How does asbestosis manifest?

A
  • Pleura: fibrosis, effusions, mesothelioma
  • Lung: interstitial fibrosis, carcinoma
  • Extrapulmonary neoplasms
37
Q

what is this?

A
  • candle wax drippings on pleura histologically showing hyalinized collagen
  • this is a pleural plaque formation in asbestosis
38
Q

Mesothelioma is associated with ?

A

Asbestos exposure, this can occur decades after initial exposure