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2 - CPR (pulmonary) > Respiratory Pathology - 3 (ILDs) > Flashcards

Respiratory Pathology - 3 (ILDs) Flashcards

(55 cards)

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1
Q

Interstitial Lung Diseases are what type of Lung Diseases?

A

Restrictive

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2
Q

What characterizes Restrictive (Interstitial) Lung Diseases?

A

DECREASED VOLUME

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3
Q

Levels of FEV1/FVC, FVC, TLC for Restrictive (Interstitial) Lung Diseases?

A 
FEV1/FVC = Normal
FVC = Decreased
TLC = Decreased
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4
Q

Idiopathic Pulmonary Fibrosis involves lung tissue being damaged in what fashion?

A

WAVES of inflammatory injury –> Fibrosis

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5
Q

With Idiopathic Pulmonary Fibrosis, what does the pathology show?

A

UIP = Usual Interstitial Pneumonia

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6
Q

What is Usual Interstitial Pneumonia and what is it seen with?

A
  • Seen with Idiopathic Pulmonary Fibrosis
    1. Normal areas
    2. Inflammation areas
    3. Fibroblast foci
    4. Peripheral honeycombing fibrosis
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7
Q

What is Usual Interstitial Pneumonia and what is it seen with?

A
  • Seen with Idiopathic Pulmonary Fibrosis
    1. Normal areas
    2. Inflammation areas
    3. Fibroblast foci
    4. Peripheral honeycombing fibrosis
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8
Q

What are some contributing factors for Idiopathic Pulmonary Fibrosis?

A

Smoking
Increasing age
Genetics

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9
Q

What are the symptoms, auscultation and X-ray findings with Idiopathic Pulmonary Fibrosis?

A
  • Dyspnea
  • Velcro-like crackles
  • Basilar infiltrates
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10
Q

Prognosis and possible treatments for Idiopathic Pulmonary Fibrosis?

A
  • Poor prognosis, death within 3-5 years
  • Possible treatments:
    1. Lung transplant
    2. Arrest Fibrosis
    • Tyrosine Kinase Inhibitors
    • TGF-beta Inhibitors
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11
Q

What autoimmune disease can manifest as Idiopathic Pulmonary Fibrosis?

A

Rheumatoid Arthritis

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12
Q

Non-specific Interstitial Pneumonia (NSIP) is also idiopathic and has a better prognosis than UIP. What is the histology?

A

UNIFORM pattern of inflammation and fibrosis

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13
Q

A UNIFORM pattern of inflammation and fibrosis is seen with?

A

NSIP (non-specific interstitial pneumonia)

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14
Q

What autoimmune disease can manifest as Non-Specific Interstitial Pneumonia (NSIP)?

A

Systemic Sclerosis

– UNIFORM inflammation and fibrosis

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15
Q

When does Cryptogenic Organizing Pneumonia (COP) occur?

A

Superimposed on prior infection/inflammatory process

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16
Q

Cryptogenic Organizing Pneumonia (COP) histology findings?

A

Fibroblast foci = plugs of loose connective tissue

masson bodies

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17
Q

Masson bodies = plugs of loose connective tissue (fibroblast foci) are seen with which ILD?

A

Cryptogenic Organizing Pneumonia (COP)

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18
Q

Cryptogenic Organizing Pneumonia is a diagnosis of ______ and patients will fully recover with?

A

Diagnosis of Exclusion!

- Patients fully recover with oral steroids

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19
Q

What autoimmune disease can manifest as Cryptogenic Organizing Pneumonia (COP)?

A

SLE

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20
Q

What are 2 Granulomatous ILDs?

A

Sarcoidosis

Hypersensitivity Pneumonitis

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21
Q

Sarcoidosis

A

Systemic disease with NON-CASEATING Granulomata in various organs

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22
Q

Non-caseating granulomata in various organs, usually involves lungs/hilar lymph nodes

A

Sarcoidosis

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23
Q

Describe the patient that usually presents with Sarcoidosis

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A

Younger Black person with elevated ACE levels and/or dyspnea

24
Q

Sarcoidosis involves granulomatous inflammation. What cells/inclusions should you look for?

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A

Giant cells

- Asteroid body or Schaumann bodies inclusions

25
What are the stages of Sarcoidosis and do they occur in order?
``` NO they do not occur in order 1 = lymphadenopathy 2 = lymphadenopathy and pulmonary infiltrate 3 = pulmonary infiltrate 4 = fibrosis ```
26
Sarcoidosis patients will usually die to what involvement?
Pulmonary Cardiac Neurologic
27
What defines Hypersensitivity Pneumonitis?
Ill-defined airway centered Granulomata
28
Ill-defined airway centered Granulomata
Hypersensitivity Pneumonitis
29
What does a diagnosis of Hypersensitivity Pneumonitis require?
HISTORY
30
Hypersensitivity Pneumonitis is an immune reaction due to?
Inhaled antigen
31
Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled protein from bird poop
Pigeon Breeder's lung
32
Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled actinomycetic spore in hay
Farmer's lung
33
Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled MAC?
Hot Tub lung
34
What are 3 smoking related ILDs?
Desquamative Interstitial Pneumonia Respiratory Bronchiolitis - ILD Langerhans Cell Histiocytosis
35
What are 3 smoking related ILDs?
Desquamative Interstitial Pneumonia (DSIP) Respiratory Bronchiolitis - ILD (RB-ILD) Langerhans Cell Histiocytosis (LCH)
36
Desquamative Interstitial Pneumonia (DSIP) involves smokers at what age and what is the treatment?
40-50s | Stop smoking and corticosteroids -- good prognosis
37
Desquamative Interstitial Pneumonia (DSIP) histology findings?
Stuffed alveolar spaces full of macrophages
38
Stuffed alveolar spaces full of macrophages is seen with?
DSIP (desquamative interstitial pneumonia) | -- smokers in 40-50s
39
Respiratory Bronchiolitis - ILD involve smokers at what age?
30-40s and less symptoms than DSIP
40
What will be seen on histology with RB-ILD?
- Less macrophages - Peribronchiolar Metaplasia (abnormally located ciliated cells) - Fibrosis in later stages
41
What will be seen on histology with Respiratory Bronchiolitis - ILD?
- Less macrophages - Peribronchiolar Metaplasia (abnormally located ciliated cells) - Fibrosis in later stages
42
If RB-ILD is caught early and with smoking cessation it can be ____
Reversible
43
What prompts a biopsy for diagnosis with RB-ILD?
Radiographic abnormalities
44
Who does Langerhans Cell Histiocytosis affect and what lesions are present?
Young smokers with stellate lesions on lungs
45
Describe how Langerhans Cell Histiocytosis (LCH) may present?
- Progressive scarring - Cysts - Cysts rupture = Pneumothorax
46
Describe how Langerhans Cell Histiocytosis (LCH) may present?
- Progressive scarring - Cysts - Cysts rupture = Pneumothorax
47
What cells/things are present on histology with Langerhans Cell Histiocytosis?
- Eosinophils - Langerhans cells (immature dendritic cells) - Fibrosis and cysts
48
What markers are (+) with Langerhans Cell Histiocytosis?
(+) s-100 and CD1a
49
Langerhans Cell Histiocytosis will reverse with?
Smoking cessation
50
(+) s-100 and CD1a
Langerhans Cell Histiocytosis
51
Pulmonary Alveolar Proteinosis (PAP)
Impairment of surfactant metabolism that causes it to accumulate throughout alveoli and airspaces
52
Impairment of surfactant metabolism that causes it to accumulate throughout alveoli and airspaces
Pulmonary Alveolar Proteinosis (PAP)
53
Pulmonary Alveolar Proteinosis is due to a defect in?
GM-CSF
54
An autoimmune defect in GM-CSF will cause?
Pulmonary Alveolar Proteinosis (PAP) | -- buildup of surfactant
55
What is treatment for Pulmonary Alveolar Proteinosis?
Subcutaneous GM-CSF

2 - CPR (pulmonary) (26 decks)

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