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RC Test 3 > B4.056 - Restrictive Lung Diseases: Interstitial Lung Disease > Flashcards

B4.056 - Restrictive Lung Diseases: Interstitial Lung Disease Flashcards

1
Q

Diseases affecting the pulmonary interstitium result in

A

Effective loss of lung tissue

decrease in lungs ability to expand

decrease in the lungs ability to transfer oxygen or CO2 within the blood

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2
Q

what are fibrosisng interstitial lung diseases

A
  1. idiopathic pulmonary fibrosis
  2. non specific interstitial pneumonia
  3. cryptogenic organizing pneumonia
  4. drugs
  5. connective tissue diseases
  6. pneumoconiosis
  7. radiation
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3
Q

what are granulomatous interstitial lung diseases

A
  1. sarcoidosis
  2. hypersensitivity penumonitis
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4
Q

what re smoking related interstitial lung diseases

A

desquamative interstitial pneumonia

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5
Q

what are other interstitial lung diseases

A

pulmonary alveolar proteinosis

langerhans cell histiocytosis/eosinophilic granulom

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6
Q

characterize idiopathic pulmonary fibrosis

A

chronic progressive fibrosing interstitial lung disease without an identifiable cause and with a high mortality rate

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7
Q

how do you diagnose idiopathic pulmonary fibrosis

A

progressive scarring of lung parenchyma and loss of lung function

characteristic clinical, pathologic and radiologic findings

typical histologic pattern is usual interstitial pneumonia (UIP)

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8
Q

how do you get IPF

A

environmental exposures

genetically predisposed prone to aberrant repair of recurrent alveolar epithelial cell injury

increased fibrogenic cytokines like TGFbeta

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9
Q

what is the epidemiology of idiopathic pulmonary fibrosis

A

older, smoker maybe

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10
Q

describe the presentation of idiopathic pulmonary fibrosis

A

insidious onset of worsening dyspnea and dry couhg

5th - 6th decade at presentation

fine bibasilar inspiratory crackles in >80% of patients, with progression upward as teh disease advances

subsequent hypoxemia, cyanosis and clubbing

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11
Q
A

cobblestoned pleural surface seen in idiopathic pulmonary fibrosis

due to fibrosis of interlobar septae

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12
Q
A

extensive fibrosis, accentuated subpleurally and in lower lobe

seen in idiopathic pulmonary fibrosis

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13
Q

what is this and what is the blue line pointing to

A

Usual interstitial pneumonia

Fibroblast focus (plump fibroblasts)

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14
Q

what is this and when is it seen

A

Left arrow - established (late) fibrosis

Right arrow - uninvolved, note subpleural area more affected

Usual interstitial pneumonia

seen in idiopathic pulmonary fibrosis

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15
Q

what is characteristic of the distribution of idiopathic pulmonary fibrosis

A

spatially and temporally hetoerogenous distribution: i.e. normal and abnormal areas, early fibrosis (fibroblast foci) and established fibrosis

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16
Q
A

UIP in IPF

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17
Q
A

end stage lung with honeycomb fibrosis

UIP in IPF

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18
Q

what is the differential for UIP microscopic pattern

A

Idiopathic pulmonary fibrosis

collagen vascular disease

drug

chronic hypersensitivity pneumonitis

asbestosis

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19
Q

course of IPF

A

chronic progressive, downhill course

may have acute exacerbations

progressive lung disease, end stage lung and/or pulmonary hypertension

increased risk of lung cancer

respiratory failure most frequent cause of death

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20
Q

what are the 2 drugs for treatment of IPF

A

pirfenidone - antifibrotic med

nintedanib - TKI inhibits fibrosis pathways

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21
Q

definitive treatment of IPF

A

transplant

post transplatn 5- yr survival about 50-60%

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22
Q

what is the etiology of cryptogenic organizing pneumonia

A

unknown!

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23
Q

what are characterstics of cryptogenic organizing pneumonia

A

polypoid plugs of loose organizing connective tissue within small airways, alveolar ducts, and the surrounding alveoli

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24
Q
A

cryptogenic organizing pneumonia

note: polypoid plugs

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25
Q
A

cryptogenic organizing pneumonia

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26
Q

what are the symptoms of cryptogenic organizing pneumonia

A

variable onset

cough

dyspnea

may sponteneously resolve

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27
Q

treatment for cryptogenic organizing pneumonia

A

steroid treatment for most patients for complete recovery

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28
Q

what is the differential for organizing pneumonia pattern

A

viral and bacterial

inhaled toxins

drugs

collagen vascular diseases

GVHD

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29
Q

what is pneumonconiosis

A

non neoplastic reaction of teh lung to inhaled mineral dust, organic particles or chemical vapros

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30
Q

what size of inspired particles can affect pneumonconiosis

A

1-5 microns they can go further down past escalator

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31
Q

what does solubility of particles affect in pneumonconiosis

A

if theyre soluble they may be able to be taken care of by other cells but if not then they may cause problems

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32
Q

what leads to disease in pneumonconiosis

A

the particles inhaled stimulate an immune resonse that leads to disease

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33
Q

what are types of pneumonconiosis

A

aspestosis

silicosis

coal workers pneumonconiosis

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34
Q

what jobs are associated with asbestos related diseases

A

minin, milling, fabrication, insulation

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35
Q

what is asbestos

A

crystalline hydrated silicates that form fibers

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36
Q

what are the asbestos amphiboles

A

all but chrysotile

anthrophyllite

crocidolite

amosite

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37
Q

what are amphibole asbestos fibers

A

less prevalent but more potent for causing diseases, especially mesothelioma

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38
Q

what is the most common asbestos fiber

A

chrysotile

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39
Q
A

chrysotile asbestos

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40
Q

what happens in asbestos related disease

A

asbestos fibers are phagocytosed by macrophages acivate the inflammasone and stimulate the release of proinflammatory and fibrogenic factors

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41
Q
A

pleural plaques associated with aspestos related pleural disease

usually asymptomatic

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42
Q

recurrent pleural effusions in patients with asbestos exposrue

A

not common, think mesothelioma

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43
Q
A

extensive interstitial fibrosis associated with asbestos exposure

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44
Q
A

asbestos body

brown indicates iron from macrophage ferritin

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45
Q

describe how asbestos relates to malignancy

A

tumor initiator and promoter

asbestos fibers generate reactive free radicals in distal lung, close to mesothelium. toxic chemicals absorbed into asbestos fibers

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46
Q

does smoking increase risk for mesothelioma

A

no just like mathey said

47
Q
A

lung cancer related to asbestos malignancy

48
Q

risk for lung cancer related to asbestos

A

5x

55x if also a smoker

49
Q

where does malignant mesothelioma arise

A

in pleura

50
Q

latent period and lifetime risk for mesothelioma with asbestos exposure

A

latent period 25-45 yrs

lifetime risk - 7-10%

51
Q

presentation of mesothelioma

A

chest pain, dyspnea, pleural effusions

grow extensively in pleural cavity, invade lungs and spread to lymph nodes, liver, distant metastatic sites

52
Q
A

epitheliod type malignant mesothelioma

53
Q
A

mixed type malignant mesothelioma

54
Q

describe the genetics of malignant mesothelioma

A

homozygous deletion of tumor suppressor gene CDKN2A/INK4a on chromosome 9p

55
Q

prognosis for mesothelioma and treatment

A

50% die in 12 months of Dx

chemo, radiaton, extrapleural pneumonectomy may help

56
Q

what is silicosis

A

a lung disease caused by inhalation of proinflammatory crystalline silicon dioxide (silica)

usually decades of exposure, rarely rapid

57
Q

most prevalent chronic occupational disease

A

silicosis

58
Q

presentation of silicosis

A

slowly progessing, nodular, fibrosing pneumoconiosis

59
Q

epidemiology of silicosis

A

african americans

foundry work, sandblasting, hardrock mining, stone cutting

60
Q

what is the most common causative inhalant in silicosis

A

crystalline quartz (more fibrogenic)

61
Q

what does silicosis lead to an increased risk of

A

TB, lung cancer

62
Q
A

silicosis

63
Q
A

silicosis

64
Q
A

silicosis

Silica particles seen under polarized light

65
Q

what is coal workers pneumoconiosis

A

parnchymal lung disease that results from the inhalation of, retention of and host respose to coal dust

66
Q

what is an anthracosis

A

innocuous coal induced pulmonary lesion in coal miners, all urban dwellers and tobacco smokers

67
Q

what happens when coal is inhaled

A

inhaled carbon pigment is engulfed by alveolar or intesrtitial macrophages, which then accumulate in connective tissue along lymphatics or in lympoid tissue

68
Q
A

complicated CWP

progressive massive fibrosis

69
Q
A

CWP

dense scars with collagen and pigment

70
Q

what is sarcoidosis

A

a multisystem disease of unkown cause

causes non necrotizing granulomas

71
Q

describe the way sarcoidosis looks on imagin

A

bilateral hilar lymphadenopathy or lung involvement is visible on chest imaging

72
Q

what other lesions are associated with sarcoidosis

A

eye and skin lesions

73
Q

pathogenesis of sarcoidosis

A

cell mediated immune response to an unidentified antigen

CD4 helper T cells

increased cytokines (TNF)

HLA-A1, HLA-B8

74
Q
A

sarcoidosis

75
Q
A

sarcoidosis

76
Q
A

sarcoidosis

77
Q
A

sarcoidosis

note: multinucleated giant cells

epitheliod histiocytes

78
Q

epidemiology of sarcoidosis

A

african americans more common

women more common

lung/hilar lymph nodes involved usually

79
Q
A

sarcoidosis

asteroid body

80
Q
A

sarcoidosis

Schaumann body

81
Q

lab values in sarcoidosis

A

elevated serum ACE

hypercalcemia or hypercalciuria leading to nephrolithiasis and renal dysfunction can occur

enzyme 1a - hydroxylase in activated macrophages that convert 25-hydroxyvitamin D to 1,26 -mdihydroxyvitamin D, the active form of the vitamin. this results in increased gut absorption

82
Q

course and treatment of sarcoidosis

A

variable

treatment: steroids for progressive/dangerous disease

65-79% of patients recover

20% have impaired eye or lung function

83
Q

hypersensitivity pneumonitis

A

a spectrum of immunologically mediated, predominantly interstitial, lung disorders caused by intense, often prolonged exposure to inhlaed organin antigens.

abnormal sensitivity to antigen, affecting primarily the alveoli

84
Q

prevention of hypersensitivity pneumonitis

A

removal of environmental agent

85
Q

BAL of hypersenstivity pneumonitis

A

increased CD4 and CD8 T lymphocytes

specific antibodies against the causative antigen in serum

complement and immunoglobulins

noncaseating granulomas in 2/3rd of patients: T cell mediated (type 4) HS againts antigens

86
Q
A

loose granulomas in HS pneumonitis

87
Q
A

granuloma and chronic inflammation in HS pneumonitis

88
Q

clinical syndrome of HS pneumonitis

A

bird fanciers disease, (protein in serum, feathers, droppings)

Farmers lung (thermophilic actinomycetes in moldy hay)

Bagassosis (moldy sugar cane)

Humidifier lung (thermophilic bacteria in heated water reservoirs)

89
Q

clinicla presentation of HS pneumonities

A

acute episodes follow inhalation of antigenic dust in a previously sensitized patient usually 4-6 hrs after exposure. Recurrent episodes of dyspnea, fevver, cough and increased white cells

90
Q

management of HS pneumonitis

A

steroids

remove anitgen

transplant if it goes on a long time and you get fibrosis

91
Q
A

drug associated interstitial disease

Belomycin

Methotrexate

amiodarone

nitrofurantoin

aspirin

beta antagonists

92
Q

what is pulmonary langerhans cell histocytosis/eosinophilic granuloma and who is it seen in

A

interstitial lung disease with proliferation of langerhans cells (immature dendritic cells)

adults

smoking

nodules or nodules with cystic change

93
Q

classic complication of langerhans histocytosis

A

pneumothorax bc its right up next to pleural space

94
Q
A

pulmonary langerhans cell histocytosis

95
Q
A

pulmonary langerhans histocytosis

96
Q
A

pulmonary langerhans histiocytosis

97
Q
A

birbeck granules tennis racket shape

langerhans histocytosis

98
Q

treatment for pulmonary langerhans histiocytosis

A

cessation of tobacco smoking, steroids, cyclophosphamide, lung transplant

99
Q

pulmonary alveolar proteinosis

A

rare

characterized by bilateral patchy asymmetric pulmonary opacification on imaging

accumulation of acellular surfactant in the intra alveolar and bronchiolar spaces

100
Q

pulmonary alveolar proteinosis commonest type

A

autoimmune aquired

anti GM-CSF signaling

loss of GM CSF signaling blocks terminal differentition of alveolar macrophages impairing their ability to catabolize surfactant

101
Q
A

alveolar proteinosis

102
Q
A

alveolar proteinsois

granular material and histiocytes within alveolar spaces

103
Q
A

lamellar bodies

alveolar proteinosis

104
Q

clinical presentation of alveolar proetinosis and management

A
105
Q
A

pulmonary hypertension

106
Q
A

honeycomb lung

107
Q
A
108
Q

a 55 yo man originally from metsova area, northwest greece presents with dyspnea, cough, fever and chest pain. Medial occupational history were unremarkable. Physical exam unremarkable. X ray showed irregular linear densities, particularly in the lower lungs, pleural effusion and pleural thickening. PFTs revealed restrictive lung function. Dx?

A

asbestosis

109
Q

60 yo with 3 year history of cough and progressive dyspnea. His PMH of cough and progressive dyspnea. Heavy smoker. Not taking any drugs, non occupational exposures. Gradual appearance of dyspnea on exertion which worsened with time and was associated with a dry cough, no pain, fever or other symptoms. Elevarted RR and bibasilar velcro rales. Cyanosis and clubbing.

A

IPF

110
Q
A

fibroblast foci

IPF

111
Q
A

HS PF

112
Q

most likey microscopic finding of HS P?

A

granuloma

113
Q

24 to African American woman presents with fatigue, ahces and pains, blurry vision, dry eyes and SOB. Small cutaneous bumps, uveitis and enlarged liver. Abnormal X rays restrictiv lung findings, increased ACE and 24 hr Ca++ excretion. Bronchioloalveolar lavage revealed CD4/8 ratio of >5

A

sacroidosis

RC Test 3 (15 decks)

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