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Respiratory > Interstitial Lung Diseases > Flashcards

Interstitial Lung Diseases Flashcards

1
Q

What are interstitial lung diseases (ILDs)?

A

A group of disorders characterized by cellular infiltration, scarring and/or architectural disruption of the pulmonary parenchyma

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2
Q

What are the ssx of ILD?

A

Dry cough with progressive DOE

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3
Q

What are PFTs like with ILDs?

A

Restrictive pattern (decreased FEV, and normal FEV1/FVC)

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4
Q

What are the CT/ CXR findings with ILDs?

A

Interstitial infiltrates and Ground glass appearance

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5
Q

What are the major occupational exposures that lead to ILD? (4)

A

Silica
Asbestos
Farms
Fumes

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6
Q

What are the two classic drugs that lead to ILD?

A

Amiodarone

Nitrofurantoin (macrobid)

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7
Q

What are the connective tissue disorders that can lead to ILD?

A

SLE
RA
Scleroderma

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8
Q

What are the signs of ILD?

A

Clubbing
Erythema/rash
Arthritis

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9
Q

What are the lungs sounds with ILD?

A

Dry crackles

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10
Q

What heart findings are found with ILD? (4)

A

Increased right heart pressure:

  • increased P2 intensity
  • TR murmur
  • edema
  • JVD
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11
Q

What is usually the first change on a PFT with ILD? Why?

A

Decreased DLCO due to non homogeneous thickening of the alveoli

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12
Q

What is usually the first change on a PFT with COPD? Why?

A

FEV/FVC ratio

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13
Q

What are the two ILDs that have increased lung volumes?

A

LAM

Langerhans histiocytosis

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14
Q

What is the granulomatous ILD?

A

Sarcoidosis

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15
Q

What are the four known association ILDs?

A

CT diseases
Drugs
Occupational exposure
Hypersensitivity

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16
Q

What are the idiopathic Interstitial pnuemonias?

A
  • Idiopathic pulmonary fibrosis
  • non-specific interstitial pneumonia
  • Cryptogenic organizing pneumonia
  • respiratory bronchiolitis
  • desquamative interstitial pneumonia
  • acute interstitial pneumonia
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17
Q

What patients with idiopathic interstitial pneumonias respond to therapy?

A

No

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18
Q

What is the prognosis (generally) for idiopathic pulmonary fibrosis?

A

Bad-3-5 year survival rate

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19
Q

What is the history commonality for idiopathic pulmonary fibrosis?

A

Smoking

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20
Q

Who usually gets idiopathic pulmonary fibrosis?

A

50-70 year old smokers

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21
Q

What are the ssx of idiopathic pulmonary fibrosis?

A

Chronic and progressive DOE, and non-productive cough

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22
Q

What are the lung sounds with idiopathic pulmonary fibrosis?

A

Bibasilar inspiratory “velcro-like” crackles

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23
Q

What percent of patients with idiopathic pulmonary fibrosis have clubbing?

A

25%

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24
Q

What causes the crackles with idiopathic pulmonary fibrosis?

A

Expanding alveoli

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25
Q

What are the CXR findings with idiopathic pulmonary fibrosis? (which lobe? central or peripheral distribution? What type of CT changes)?

A

Lower lobe and peripheral distribution of reticular infiltrates and fibrotic changes

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26
Q

What are the CT changes with idiopathic pulmonary fibrosis?

A

Patchy honeycombing and traction bronchiectasis

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27
Q

What are the tissue changes that can be seen in with idiopathic pulmonary fibrosis?

A

Usual interstitial pneumonitis

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28
Q

What are the drugs used to treat idiopathic pulmonary fibrosis? MOA?

A

Pirfenidone
Nintedanib

Inhibit IL-1beta and TNF-alpha from human lung fibroblasts

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29
Q

What is the MOA of etanercept? Is this effective in idiopathic pulmonary fibrosis?

A

Anti-TNF antibody

Not effective

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30
Q

What is N-acetylcysteine used for usually? Is it effective for idiopathic pulmonary fibrosis?

A

Acetaminophen overdose

Not effective

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31
Q

What is the MOA of Pirfenidone? Use?

A

Inhibit IL-1beta and TNF-alpha from human lung fibroblasts

Treats idiopathic pulmonary fibrosis

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32
Q

What is the MOA of nintedanib? Use?

A

Inhibit IL-1beta and TNF-alpha from human lung fibroblasts

Treats idiopathic pulmonary fibrosis

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33
Q

What are nonspecific interstitial pneumonitis (NSIP)?

A

Homogenic CT disease of the lungs

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34
Q

What is the prognosis (generally) with interstitial pneumonitis?

A

better than IPF

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35
Q

What are the imaging findings with nonspecific interstitial pneumonitis (NSIP)?

A

Ground glass appearance symmetrically in the lower lung fields

36
Q

Which disease is honeycombing common in: idiopathic pulmonary fibrosis or nonspecific interstitial pneumonitis (NSIP)?

A

idiopathic pulmonary fibrosis

37
Q

What is the therapy for nonspecific interstitial pneumonitis (NSIP)?

38
Q

What are the two ILDs that are definitively linked to smoking?

A

Desquamative interstitial pneumonitis (DIP)

Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)

39
Q

What are the histological characteristics of Desquamative interstitial pneumonitis (DIP) and Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)?

A

Peribronchiolar accumulation of pigmented macrophages

40
Q

What is the treatment for Desquamative interstitial pneumonitis (DIP) and Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)?

A

Stop smoking

Steroids

41
Q

What are the imaging findings with DIP and RB-ILD?

A

Diffuse, patchy areas of ground glass appearance

42
Q

What is acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)

A

Sudden and fulminant form of diffuse lung injury that generally occurs in previously healthy individuals

43
Q

What are the ssx ofacute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)

A

Fever
Cough
SOB

44
Q

What is the prognosis for acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?

A

50% mortality, and those that survive develop fibrosis

45
Q

What are the imaging findings with acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?

A

Diffuse, bilateral air space and reticular infiltrates

46
Q

What is the treatment for acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?

A

Supportive

47
Q

What is cryptogenic organizing pneumonia (COP)?

A

a non-infectious pneumonia; specifically, an inflammation of the bronchioles (bronchiolitis[1]) and surrounding tissue in the lungs.

48
Q

Who usually gets cryptogenic organizing pneumonia (COP)?s

49
Q

What is the clinical presentation of cryptogenic organizing pneumonia (COP)?

A

LRTI with lack of response to abx

50
Q

LRTI with lack of response to abx = ?

A

cryptogenic organizing pneumonia (COP)

51
Q

What is the treatment and prognosis for cryptogenic organizing pneumonia (COP)?

A

Corticosteroids

Good prognosis

52
Q

What is the best way to diagnose cryptogenic organizing pneumonia (COP)? (2)

A

Bronchoscopy

Alveolar lavage

53
Q

What are the imaging findings of cryptogenic organizing pneumonia (COP)? Where are they in the lungs?

A

Patchy infiltrates (unilaterally or bilaterally), with small nodular opacities

Usually subpleural

54
Q

What is hypersensitivity pneumonitis?

A

Repeated exposure and immunologic sensitization to organic antigens

55
Q

What are the risk factors for developing hypersensitivity pneumonitis? (3)

A

Bird feathers
Thermophilic bacteria
Farmers

56
Q

What are the cells that mediate hypersensitivity pneumonitis? What do these do?

A

CD8 +
Macrophages

Granuloma formation

57
Q

What is the usual acute presentation of hypersensitivity pneumonitis?

A

Abrupt onset of fever, chills, nausea, cough, chest tightness and dysponea

58
Q

What are the PE findings with hypersensitivity pneumonitis?

A

Tachypnea

Diffuse, fine crackles

59
Q

What are the imaging findings of hypersensitivity pneumonitis?

A

Normal to patch, micronodular infiltrates

60
Q

What is the treatment for hypersensitivity pneumonitis?

A

Remove antigen exposure

61
Q

What is the usual SUBacute presentation of hypersensitivity pneumonitis? Treatment?

A

Gradual development of cough, SOB, weight loss

Corticosteroids

62
Q

What is the usual presentation of CHRONIC hypersensitivity pneumonitis?

A

Insidious onset of cough, fatigue, SOB

Clubbing

63
Q

What is the major complication of chronic hypersensitivity pneumonitis?

A

Irreversible Pulmonary fibrosis

64
Q

What are the CT findings with hypersensitivity pneumonitis? (2)

A

Ground glass and centrilobular nodules

65
Q

Why should you r/o other causes of lung disease prior to treating any of the ILDs?

A

Steroids will weaken the immune response, which will be bad with infectious causes

66
Q

What is the lung disease that amiodarone causes? Who usually gets this?

A

Interstitial pneumonitis

Patients who are on the drug long term

67
Q

What are the imaging findings with amiodarone toxicity? (2)

A

Ground glass appearance, with increased attenuation of the liver/spleen

68
Q

What are the two presentations of lung toxicity induced by nitrofurantoin?

A

Acute after course of abx

Chronic after several months of abx

69
Q

What are the peripheral blood findings with lung toxicity from nitrofurantoin?

A

Eosinophilia

70
Q

What are the cytokines that are elevated with nitrofurantoin lung toxicity?

A

Serum gamma globulin
Serum transaminases
ANA

71
Q

What is acute eosinophilic pneumonia? What causes it?

A

Acute febrile illness of 1-4 weeks duration with usual symptoms.

Associated with resumption of smoking, or inhalation exposure

72
Q

What are the peripheral blood smear findings with acute eosinophilic pneumonia?

73
Q

What are the histological findings with acute eosinophilic pneumonia?

A

Extensive eosinophilic infiltration in the interstitium

74
Q

What are the CT findings of acute eosinophilic pneumonia?

A

Bilateral ground glass

75
Q

Which is more likely to have eosinophilia: chronic or acute eosinophilic pneumonia?

76
Q

What is chronic eosinophilic pneumonia?

A

Idiopathic infiltration of eosinophils in the interstitial or alveolar spaces

77
Q

What are the imaging findings of chronic eosinophilic pneumonia

A

bilateral peripheral pulmonary edema

“negative of pulmonary edema”

78
Q

What are the peripheral blood smear findings with chronic eosinophilic pneumonia?

A

High levels of eosinophils

79
Q

What is lymphangioleiomyomatosis? Who is affected?

A

Progressive cystic lung disease that occurs in women of childbearing years

80
Q

What are the histological findings of lymphangioleiomyomatosis?

A

Proliferation of atypical smooth muscle cells

81
Q

What are the ssx of lymphangioleiomyomatosis?

A

DOE
Hemoptysis
Pneumothorax

82
Q

What is the usual presenting sign of lymphangioleiomyomatosis?

A

Pneumothorax

83
Q

What is the treatment for lymphangioleiomyomatosis?

A

Lung transplant

84
Q

What is the best test to get to differentiate ILDs?

A

High res CT (HRCT)

85
Q

Idiopathic pulmonary fibrosis and non-specific interstitial pneumonitis are very similar. What are the major differences between the two? (hint: age, CT findings, prognosis, pathological pattern)?

A

IPF = older smokers, honeycombing, bad prognosis, UIP

NSIP: Younger pts, non-honeycombing, better prognosis, temporal heterogeneity pattern,

Respiratory (34 decks)

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