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

Respiratory Pathology - 3 (ILDs) Flashcards

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

A

Younger Black person with elevated ACE levels and/or dyspnea

24
Q

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

A

Giant cells

- Asteroid body or Schaumann bodies inclusions

25
Q

What are the stages of Sarcoidosis and do they occur in order?

A 
NO they do not occur in order
1 = lymphadenopathy
2 = lymphadenopathy and pulmonary infiltrate
3 = pulmonary infiltrate
4 = fibrosis
26
Q

Sarcoidosis patients will usually die to what involvement?

A

Pulmonary
Cardiac
Neurologic

27
Q

What defines Hypersensitivity Pneumonitis?

A

Ill-defined airway centered Granulomata

28
Q

Ill-defined airway centered Granulomata

A

Hypersensitivity Pneumonitis

29
Q

What does a diagnosis of Hypersensitivity Pneumonitis require?

A

HISTORY

30
Q

Hypersensitivity Pneumonitis is an immune reaction due to?

A

Inhaled antigen

31
Q

Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled protein from bird poop

A

Pigeon Breeder’s lung

32
Q

Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled actinomycetic spore in hay

A

Farmer’s lung

33
Q

Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled MAC?

A

Hot Tub lung

34
Q

What are 3 smoking related ILDs?

A

Desquamative Interstitial Pneumonia
Respiratory Bronchiolitis - ILD
Langerhans Cell Histiocytosis

35
Q

What are 3 smoking related ILDs?

A

Desquamative Interstitial Pneumonia (DSIP)
Respiratory Bronchiolitis - ILD (RB-ILD)
Langerhans Cell Histiocytosis (LCH)

36
Q

Desquamative Interstitial Pneumonia (DSIP) involves smokers at what age and what is the treatment?

A

40-50s

Stop smoking and corticosteroids – good prognosis

37
Q

Desquamative Interstitial Pneumonia (DSIP) histology findings?

A

Stuffed alveolar spaces full of macrophages

38
Q

Stuffed alveolar spaces full of macrophages is seen with?

A

DSIP (desquamative interstitial pneumonia)

– smokers in 40-50s

39
Q

Respiratory Bronchiolitis - ILD involve smokers at what age?

A

30-40s and less symptoms than DSIP

40
Q

What will be seen on histology with RB-ILD?

A
  • Less macrophages
  • Peribronchiolar Metaplasia
    (abnormally located ciliated cells)
  • Fibrosis in later stages
41
Q

What will be seen on histology with Respiratory Bronchiolitis - ILD?

A
  • Less macrophages
  • Peribronchiolar Metaplasia
    (abnormally located ciliated cells)
  • Fibrosis in later stages
42
Q

If RB-ILD is caught early and with smoking cessation it can be ____

A

Reversible

43
Q

What prompts a biopsy for diagnosis with RB-ILD?

A

Radiographic abnormalities

44
Q

Who does Langerhans Cell Histiocytosis affect and what lesions are present?

A

Young smokers with stellate lesions on lungs

45
Q

Describe how Langerhans Cell Histiocytosis (LCH) may present?

A
  • Progressive scarring
  • Cysts
  • Cysts rupture
    = Pneumothorax
46
Q

Describe how Langerhans Cell Histiocytosis (LCH) may present?

A
  • Progressive scarring
  • Cysts
  • Cysts rupture
    = Pneumothorax
47
Q

What cells/things are present on histology with Langerhans Cell Histiocytosis?

A
  • Eosinophils
  • Langerhans cells (immature dendritic cells)
  • Fibrosis and cysts
48
Q

What markers are (+) with Langerhans Cell Histiocytosis?

A

(+) s-100 and CD1a

49
Q

Langerhans Cell Histiocytosis will reverse with?

A

Smoking cessation

50
Q

(+) s-100 and CD1a

A

Langerhans Cell Histiocytosis

51
Q

Pulmonary Alveolar Proteinosis (PAP)

A

Impairment of surfactant metabolism that causes it to accumulate throughout alveoli and airspaces

52
Q

Impairment of surfactant metabolism that causes it to accumulate throughout alveoli and airspaces

A

Pulmonary Alveolar Proteinosis (PAP)

53
Q

Pulmonary Alveolar Proteinosis is due to a defect in?

A

GM-CSF

54
Q

An autoimmune defect in GM-CSF will cause?

A

Pulmonary Alveolar Proteinosis (PAP)

– buildup of surfactant

55
Q

What is treatment for Pulmonary Alveolar Proteinosis?

A

Subcutaneous GM-CSF

2 - CPR (pulmonary) (26 decks)

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