Restrictive Lung Disease - Path, Gupta Flashcards

1
Q

definition of restrictive lung dz - signs, symptoms

A

heterogenous group of dz characterized by dyspnea and reduced TLC

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2
Q

what is the buzzword for the appearance of end stage lung dz in restrictive dz

A

honeycomb

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3
Q

what disease is a result of repeated cycles of injury caused by a yet unidentified agent resulting in diffuse fibrosis?

A

Idiopathic Pulmonary Fibrosis

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4
Q

presentation of idiopathic pulmonary fibrosis

A

insidious, DOE, dry cough

gradualy deteriorating course

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5
Q

treatment for IPF?

A

none current

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6
Q

how do you diagnose IPF?

A

by exclusion - you must rule out secondary causes of fibrosis such as drugs and radiation

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7
Q

what is the prognosis of IPF?

A

bad

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8
Q

in IPF, what is the appearance of the fibrosis? where is it found?

A

patchy interstitial fibrosis
predominantly lower lobe, subpleural distribution
will have areas of normal lung tissue intervening
**It is heterogenous in time and space - different areas of damage are at a different stage in the dz process than other areas

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9
Q

what are some key microscopic findings in IPF

A

fibroblast foci, collagenized areas, mild lymphocytic inflammation

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10
Q

what is the pathogenesis of IPF? - what is the key player in fibrosis

A

TGF-beta - from injured penumocytes induces fibrosis

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11
Q

in IPF, where is the concentration of fibrosis the highest - what is its distribution?

A

subpleural - towards periphery

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12
Q

what happens to the epithelium of bronchi in IPF

A

metaplastic change - goes from more PSSCE to squamous epithelium

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13
Q

for IPF, when you look at it histologically, what is it called?

A

usual interstitial pneumonia (UIP)

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14
Q

if you see an intraalveolar pattern of fibrosing, rather than interstitial, what dz are you thinking?

A

cryptogenic organizing pneumonia

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15
Q

in cryptogenic organizing pneumonia, compare the age of the various areas of organizing connective tissue

A

all of the same age (diff from IPF)

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16
Q

what is the treatment for cryptogenic organizing pneumonia

A

steroids

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17
Q

what must you differentiate cryptogenic organizing pneumonia from? (i.e. what dz process has a similar histologic pattern)

A

resolving acute lung injury/ infection - like pneumonia, or ARDS

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18
Q

what is a defining histologic finding in cryptogenic organzing pneumonia?

A

intraalveolar plugs of loose fibroconnective tissue

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19
Q

what is the name of a non-neoplastic lung reaction to inhaled dust, chronic exposure

A

pneumoconioses

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20
Q

what are 4 examples of dusts that can cause pneumoconioses

A

coal
silica
asbestos
beryllium

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21
Q

define anthracosis

what dz process do you see them in

A

Innocuous inhaled carbonaceous pigment engulfed by macrophages that accuulates in connective tissue along lymphatics and in lymphoid tissue
seen in Coal Workers Pneumoconiosis

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22
Q

in simple CWP, what is the pulmonary function? where do you find it in the lung?

A

little/no pulmonary dysfunction

upper lobe predominant

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23
Q

what is the pathogenesis of complicated CWP, and what is the lung function?

A

there is a progression from simple to complicated CWP over many years
leads to progressive massive fibrosis
will compromise lung function

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24
Q

what is histologic finding of complicated CWP

A

black scars composed of pigment and dense collagen

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25
Q

what is a high yield association for complicated CWP - another dz

A

rheumatoid arthritis

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26
Q

what is epidemiology associations for silicosis (pneumoconiosis caused by inhalation of silica)

A

sandblasters, mine workers, stone cutters

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27
Q

what is the histologic pathology of silicosis, and where in lung do you find it?

A

nodular fibrosis, mostly in upper lobes and hilar nodes

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28
Q

what is the specific pathophys of silicosis - i.e. what causes the fibrosis

A

silica ingestion by macrophages causes release of fibrogenic mediators - results in hard collagenous nodules

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29
Q

what is a diagnostic technique you can use to visualize silica in silicosis

A

polarizing microscopy demonstrates birefringent silica particles

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30
Q

what does silicosis increase your susceptibility to and why

A

TB

likely related to impaired function of macrophages

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31
Q

for pneumoconiosis due to Beryllium (Berylliosis?) - what is the histopathologic finding and where in what tissues do you see it

A

noncaseating granulomas

seen in lung, hilar lymph nodes and systemic organs

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32
Q

what does berylliosis leave you at increased risk for

A

lung cancer

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33
Q

taking a good pt history is necessary to differentiate what disease from berylliosis?

A

sarcoidosis
also has noncaseating granulomas in lung and systemic organs
(look at question stem for epidemiology shit)

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34
Q

besides being a beryllium miner (damn unions), what other high yield epidemiology is associated with berylliosis

A

space industry

Like Jeezy said, me I’m in a spaceship, that’s right I work for NASA

35
Q

name the 4 non-neoplastic asbestos-related diseases

A

1) diffuse pleuarl fibrosis
2) benign pleural effusions
3) pleural plaques
4) parenchymal interstitial fibrosis (Asbestosis)

36
Q

what are the two types (shapes) of asbestos fibers, and which one is more harmful

A

1) Serpentine - curly flexible fibers

2) Amphibole - straight stiff fibers - more pathogenic, less easily ingested by macrophages

37
Q

what are pleural plaques (result of asbestos) and where are they found anatomically

A

circumscribed plaques of dense collagen

most often arise on parietal pleura and diphragmatic domes

38
Q

Parenchymal Interstitial Fibrosis (Asbestosis) - what is the time frame and pathogenesis - including what parts of the lung/airway

A

slowly progressive, heavy prolonged asbestos exposure, latency period 20+ years
initial injury to respiratory bronchioles and alveolar ducts, macrophages attempt to ingest and clear debris, release fibrogenic mediators - fibrosis of adjacent alveoli

39
Q

How do you differentiate Asbestosis from usual interstitial pneumonia (fibrosis) - upon looking at a histo slide

A

presence of asbestos bodies
involve the body coating asbestos fibers with iron stuff
golden brown beaded rods w/ translucent fiber cores (this will be question)

40
Q

what is a method you can use to better determine if asbestos is present in a histo slide

A

prussian blue stain
stains the iron containing proteinaceous material surrounding the asbestos fibers (ferrigenous bodies)
(know this also)
good pt history is impt to know to try this stain

41
Q

what 2 drugs do you need to rule out as causes of fibrosis in the lung

A

1) Bleomycin

2) Amiodarone

42
Q

what other therapeutic process can causes fibrosis in the lungs

A

radiation,

especially to the chest

43
Q

what are features of a histo slide containing drug induced penumonitis

A

mild interstitial fibrosis
lymphocyte inflammation
penumocyte hyperplasia

44
Q

what is the defining characteristic of sarcoidosis

A

non-necrotizing granulomatous inflammation

systemic disease of unknown cause

45
Q

what areas are affected by sarcoidosis (3)

A

hilar lymph nodes
lungs
skin

46
Q

what are some signs/symptoms of saroidosis

A

insidious onset of SOB
cough
and/or constitutional symptoms

47
Q

what is treatment for sarcoidosis

A

steroids

48
Q

what is typical epidemiology of sarcoidosis

A

black female

49
Q

sarcoidosis - what does BAL fluid show - for inflammatory cell types

A

increased CD4:CD8 ratio, greater than 2.5

due to inc CD4 (helper) T lymphocytes

50
Q

just know that sarcoidosis is disease of exclusion

A

good good

51
Q

buzzword for histopathology of sarcoidosis

A

non-caseating epitheliod granulomas
(Must know)
presence of giant cells

52
Q

sarcoidosis - what is the buzzword for the common giant cell inclusions (2)
how specific are they for sarcoidosis?

A

asteroid bodies
Schaumann bodies - laminated calcified concretions
not very specific

53
Q

what is the name for an immunologically mediated lung disorder due to prolonged exposure to inhaled organic dusts

A

hypersensitivity pneumonitis

54
Q

what type of hypersensitivity is hypersensitivity pneumonitis (chronic form)?

A

type IV (delayed type)

55
Q

for hypersensitivity pneumonitis, what leads to fibrosis? how can you prevent fibrosis?

A

chronic exposure to inhaled antigen leads to fibrosis

removal of environmental antigen prevents progression to fibrosis

56
Q

what is the buzzword for epidemiology for hypersensitivity pneumonitis

A

pigeon breeder’s disease

i.e. exposure to inhaled antigens from birds

57
Q

what is another (not birds) buzzword epidemiology for hypersensitivity pneumonitis

A

farmer’s lung

from moldy hay

58
Q

what are 3 histopathologic features of hypersensitivity pneumonitis

A
airway-centered interstitial lymphocytic inflammation 
loosely formed ill-define granulomas 
interstitial fibrosis 
(not eosionphils)
59
Q

what is the end stage microscopic appearance of hypersensitivity pneumonitis

A

honeycomb

60
Q

Desquamative Interstitial Pneumonia (DIP) - classified as what type of interstitial disease

A

smoking-related interstitial disease

61
Q

What are the 2 histopathologic features we need to know regarding desquamative interstitial pneumonia

A

1) temporal and spacial uniformity - everything everywhere looks the same on slide
2) alveolar spaces are completely full with macrophages that have a golden brown color

62
Q

what disease is associated with a BRAF mutation

A

Langerhans Cell Histiocytosis

63
Q

what is the epidemiology of Pulmonary Langerhans Cell Histiocytosis

A

95% young smokers

64
Q

what is histopathologic finding with Pulmonary Langerhans Cell histiocytosis

A

coffee bean shaped cells

and eosinophils

65
Q

what is the pathogenesis and histopathology of Pulmonary Alveolar Proteinosis

A

defect in macrophages that causes accumulation of surfactant in the alveolar spaces
alveoli are filled with dense, amorphous, protein-lipid granular precipitate
alveolar walls are normal

66
Q

what other pathology appears like Pulmonary Alveolar Proteinosis, but looks more fluffy and is caused by a fungus

A

Pneumocystis pneumonia
causative organism pneumocystis jirovecii
histo - looks more fluffy, bubbly, whatever (the shit accumulated in the alveoli)

67
Q

what stain do you use for pneumocystis and what is the appearance

A

Gomori silver stain

look like crushed ping pong balls

68
Q

what pts are susceptible to pneumocystis

A

immunocompromised hosts

69
Q

Muthiah - what is the most common cause of idiopathic interstitial pneumonia

A

usual interstitial pneumonia AKA idiopathic pulmonary fibrosis

70
Q

Muthiah - what is the most common interstitial lung disease with no know cause

A

IPF / UIP

71
Q

Muthiah - for UIP / IPF - what is the histo apperance with regards to age and distribution

A

there is injury happening at different times

72
Q

Muthiah - what is the location of IPF / UIP w/in the lung (lobe, and space)

A

lower lobe

subpleural

73
Q

Muthiah - what is the common pt w/ IPF

A

caucasian male, smoker, 6th or 7th decade

74
Q

Muthiah - UIP can be seen in many other diseases besides IPF - what makes UIP in IPF different

A

IPF have a progressively worsening course

75
Q

Muthiah - what is another pathology that can be associated with IPF and what environmental exposure

A

GERD

Wood dust exposure

76
Q

Muthiah - what antibody is associated with Rheumatoid arthritis

A

Rheumatoid factor Anti-CCP

77
Q

Muthiah - what antibody is associated with systemic sclerosis

A

Scl 70

78
Q

Muthiah - what antibody is associated with mixed connective tissue disease

A

anti-ribonucleoprotein (RNP)

79
Q

Muthiah - antibody associated with dermatomyositis / polymyositis

A

anti - Jo-1

80
Q

Muthiah - what is the most common cause of interstitial lung disease overall? which ones do we need to remember? (2)

A

hypersensitivity pneumonitis
we probably miss half the diagnoses
bird fancier’s disease
farmer’s lung

81
Q

Muthiah - how to tell apart berylliosis and sarcoidosis

A

Berylliosis - exposure to beryllium

aerospace industry, ceramic tile workers, beryllium miners

82
Q

Muthiah - does asbestos exposure cause more mesothelioma or bronchogenic carcinoma?

A

bronchogenic carcinoma

83
Q

Muthiah - what 3 drugs can cause pulmonary fibrosis

A

bleomycin, amiodarone, methotrexate

84
Q

Muthiah - what are key factors for silicosis

1) type of lung disease
2) lobe predominance
3) exposure to what/how

A

1) interstitial
2) upper lobe predominant
3) silica - sandblasters, stone cutters