Interstitial Lung Diseases Flashcards Preview

Respiratory > Interstitial Lung Diseases > Flashcards

Flashcards in Interstitial Lung Diseases Deck (85):
1

What are interstitial lung diseases (ILDs)?

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

2

What are the ssx of ILD?

Dry cough with progressive DOE

3

What are PFTs like with ILDs?

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

4

What are the CT/ CXR findings with ILDs?

Interstitial infiltrates and Ground glass appearance

5

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

Silica
Asbestos
Farms
Fumes

6

What are the two classic drugs that lead to ILD?

Amiodarone
Nitrofurantoin (macrobid)

7

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

SLE
RA
Scleroderma

8

What are the signs of ILD?

Clubbing
Erythema/rash
Arthritis

9

What are the lungs sounds with ILD?

Dry crackles

10

What heart findings are found with ILD? (4)

Increased right heart pressure:
-increased P2 intensity
-TR murmur
-edema
-JVD

11

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

Decreased DLCO due to non homogeneous thickening of the alveoli

12

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

FEV/FVC ratio

13

What are the two ILDs that have increased lung volumes?

LAM
Langerhans histiocytosis

14

What is the granulomatous ILD?

Sarcoidosis

15

What are the four known association ILDs?

CT diseases
Drugs
Occupational exposure
Hypersensitivity

16

What are the idiopathic Interstitial pnuemonias?

-Idiopathic pulmonary fibrosis
-non-specific interstitial pneumonia
-Cryptogenic organizing pneumonia
-respiratory bronchiolitis
-desquamative interstitial pneumonia
-acute interstitial pneumonia

17

What patients with idiopathic interstitial pneumonias respond to therapy?

No

18

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

Bad-3-5 year survival rate

19

What is the history commonality for idiopathic pulmonary fibrosis?

Smoking

20

Who usually gets idiopathic pulmonary fibrosis?

50-70 year old smokers

21

What are the ssx of idiopathic pulmonary fibrosis?

Chronic and progressive DOE, and non-productive cough

22

What are the lung sounds with idiopathic pulmonary fibrosis?

Bibasilar inspiratory "velcro-like" crackles

23

What percent of patients with idiopathic pulmonary fibrosis have clubbing?

25%

24

What causes the crackles with idiopathic pulmonary fibrosis?

Expanding alveoli

25

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

Lower lobe and peripheral distribution of reticular infiltrates and fibrotic changes

26

What are the CT changes with idiopathic pulmonary fibrosis?

Patchy honeycombing and traction bronchiectasis

27

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

Usual interstitial pneumonitis

28

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

Pirfenidone
Nintedanib

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

29

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

Anti-TNF antibody

Not effective

30

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

Acetaminophen overdose

Not effective

31

What is the MOA of Pirfenidone? Use?

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

Treats idiopathic pulmonary fibrosis

32

What is the MOA of nintedanib? Use?

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

Treats idiopathic pulmonary fibrosis

33

What are nonspecific interstitial pneumonitis (NSIP)?

Homogenic CT disease of the lungs

34

What is the prognosis (generally) with interstitial pneumonitis?

better than IPF

35

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

Ground glass appearance symmetrically in the lower lung fields

36

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

idiopathic pulmonary fibrosis

37

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

Steroids

38

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

Desquamative interstitial pneumonitis (DIP)

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

39

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

Peribronchiolar accumulation of pigmented macrophages

40

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

Stop smoking
Steroids

41

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

Diffuse, patchy areas of ground glass appearance

42

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

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

43

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

Fever
Cough
SOB

44

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

50% mortality, and those that survive develop fibrosis

45

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

Diffuse, bilateral air space and reticular infiltrates

46

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

Supportive

47

What is cryptogenic organizing pneumonia (COP)?

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

48

Who usually gets cryptogenic organizing pneumonia (COP)?s

Smokers

49

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

LRTI with lack of response to abx

50

LRTI with lack of response to abx = ?

cryptogenic organizing pneumonia (COP)

51

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

Corticosteroids
Good prognosis

52

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

Bronchoscopy
Alveolar lavage

53

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

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

Usually subpleural

54

What is hypersensitivity pneumonitis?

Repeated exposure and immunologic sensitization to organic antigens

55

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

Bird feathers
Thermophilic bacteria
Farmers

56

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

CD8 +
Macrophages

Granuloma formation

57

What is the usual acute presentation of hypersensitivity pneumonitis?

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

58

What are the PE findings with hypersensitivity pneumonitis?

Tachypnea
Diffuse, fine crackles

59

What are the imaging findings of hypersensitivity pneumonitis?

Normal to patch, micronodular infiltrates

60

What is the treatment for hypersensitivity pneumonitis?

Remove antigen exposure

61

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

Gradual development of cough, SOB, weight loss

Corticosteroids

62

What is the usual presentation of CHRONIC hypersensitivity pneumonitis?

Insidious onset of cough, fatigue, SOB

Clubbing

63

What is the major complication of chronic hypersensitivity pneumonitis?

Irreversible Pulmonary fibrosis

64

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

Ground glass and centrilobular nodules

65

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

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

66

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

Interstitial pneumonitis

Patients who are on the drug long term

67

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

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

68

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

Acute after course of abx

Chronic after several months of abx

69

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

Eosinophilia

70

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

Serum gamma globulin
Serum transaminases
ANA

71

What is acute eosinophilic pneumonia? What causes it?

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

Associated with resumption of smoking, or inhalation exposure

72

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

None

73

What are the histological findings with acute eosinophilic pneumonia?

Extensive eosinophilic infiltration in the interstitium

74

What are the CT findings of acute eosinophilic pneumonia?

Bilateral ground glass

75

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

Chronic

76

What is chronic eosinophilic pneumonia?

Idiopathic infiltration of eosinophils in the interstitial or alveolar spaces

77

What are the imaging findings of chronic eosinophilic pneumonia

bilateral *peripheral* pulmonary edema

("negative of pulmonary edema")

78

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

High levels of eosinophils

79

What is lymphangioleiomyomatosis? Who is affected?

Progressive cystic lung disease that occurs in women of childbearing years

80

What are the histological findings of lymphangioleiomyomatosis?

Proliferation of atypical smooth muscle cells

81

What are the ssx of lymphangioleiomyomatosis?

DOE
Hemoptysis
Pneumothorax

82

What is the usual presenting sign of lymphangioleiomyomatosis?

Pneumothorax

83

What is the treatment for lymphangioleiomyomatosis?

Lung transplant

84

What is the best test to get to differentiate ILDs?

High res CT (HRCT)

85

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)?

IPF = older smokers, honeycombing, bad prognosis, UIP

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