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Resp II > Interstitial lung diseases > Flashcards

Interstitial lung diseases Flashcards

1
Q

usual interstitial pneumonia

A

patchy ares of parenchymal fibrosis and interstitial inflammation interspersed btwn normal areas
honeycombing
most important in this category-. idiopathic pulmonary fibrosis

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2
Q

honeycombing

A

cystic airspace from retraction of surrounding fibrotic tissue

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3
Q

desquamative interstitial pneumonia

A

more homogenous than UIP
large number of intra-alveolar mononuclear cells
less prominent inflammation and little associated fibrosis
uniform process
minimal architectural distortion
pigmented macros secondary to smoking
smoking important underlying cause

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4
Q

Respiratory bronchiolitis interstitial lung disease

A 
related to DIP
associated w/pigmented macros 
interstitial inflammation not present
almost always a smoke
smoking cessations
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5
Q

non-specific interstitial penumonia

A 
mononuclear cell infiltration w/in alveolar walls
uniforma process
fribrosis variable, but less the UIP
idiopathic or CT disorder
better prognosis
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6
Q

acute interstitial pneumonia

A

organizing or fibrotic stage of alveolar damage- pattern seen in ARDS
no initial trigger identified
histo-fibroblast proliferation and type II pneumocyte hyperplasia

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7
Q

cytogenic organizing penumonia

A 
organizing fibrosis (granulation tissue) in small airways
mild degree of chronic interstitial inflammation
intraluminal airway involvement key
idiopathic
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8
Q

cytogenic organizing penumonia Ddx

A

infections, toxic inhalants, or CT disease

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9
Q

idiopathic pulmonary fibrosis

A 
dysregulated pattern of fibrosis due to epi injury
50-70yrs
insidious onset
dyspnea is most common complaint
rales on lung exam
clubbing
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10
Q

idiopathic pulmonary fibrosis CXR

A

interstitial pattern that is generally bilateral and relatively diffuse
more prominent at bases and periphery

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11
Q

idiopathic pulmonary fibrosis high resolution CT

A

interstital densities that are patchy, peripheral, subpleural and associated w/small cystic spaces
honeycombing- irreversible damage

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12
Q

Dx idopathic pulmonary fibrosis

A

surgical lung biopsy

HRCT w/classic pattern of honeycombing diagnostic in patient too frail for lung biopsy

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13
Q

idiopathic pulmonary fibrosis prognosis

A

poor w/mean survival 2-5yrs

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14
Q

desquamative interstitial pneumonia

A 
smokers
subacute
ground glass appearance on imaging
lung biopsy w/uniform accumulation of intraalveolar macros w/little or no fibrosis
prognosis better than IPF
may respond to corticosteroids
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15
Q

nonspecific interstitial pneumonia

A

ground glass due to inflammation

lung biopsy- inflammatory response in alveolar walls w/little fibrosis

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16
Q

nonspecific interstitial pneumonia prognosis

A

depends on degree of fibrosis

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17
Q

nonspecific interstitial pneumonia Tx

A

corticosteroids

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18
Q

cryptogenic organizing pneumonia

A

CT plug in small airway accompanied by mononuclear cell infiltration of surrounding lung parenchymal
subacute presentation over weeks to months w/constititional as well as respiratory symptoms

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19
Q

cryptogenic organizing pneumonia CXR

A

mimics pneumonia w/one or more alveolar infiltrates

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20
Q

cryptogenic organizing pneumonia Tx

A

response to steroids is dramatic and occurs over days to weeks
therapy is usually prolonged for months to prevent relapse

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21
Q

acute interstitial pneumonia

A

acute disease that begins w/clinical picture of ARDS, but w/o inciting cause
Histo- diffuse alveolar damage, often showing some organization and fibrosis
mortality is high

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22
Q

acute interstitial pneumonia CXR

A

ground glass

alveolar filling

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23
Q

complicating CT diseases

A 
RA
SLE
progressive systemic sclerosis (scleroderma)
polymyositis-dermatomyositis
Sjogrens syndrome
overlap syndromes
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24
Q

CT disorders

A

usually patients have evidence of underlying disease first, but lung disease can proceed diagnosis
often UIP, but sometimes NSIP or COP
lower lobe more common then upper lobe disease

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25
Q

RA

A

joints
pleura
lungs- nodules or interstitial lung disease (usually mild)

26
Q

SLE

A

joints, skin, organs
pleural disease
acute pneumonitis- alveolar walls and spaces
chronic interstitial lung disease

27
Q

progressive systemic sclerosis

A 
aka scleroderma
skin, small blood vessels
organs
scarring of pulmonary parenchyma 
pulmonary fibrosis associated a/Ab to topoisomerae I
pulmonary artery HTN
28
Q

polymyositis dermatomyositis

A

mm and skin are priamry sites of inflammatory process
interstitial lung disease uncommon
respiratory difficulty secondary to diaphragm weakness

29
Q

pulmonary langerhans histocytosis

A

aka eosinophilic granuloma of lung
histioctic cell appears to be an Ag presenting dendritic or phagocytic cell called langerhans cell
x-bodies (rod like)
histiocytes, eos, lymphocytes, macros, plasma cells

30
Q

pulmonary langerhans histocytosis clinical

A

starts as peribronchiolar distribution -> diffuse
smokers
unexplained interstitial disease in young or middle aged

31
Q

pulmonary langerhans histocytosis CXR

A

nodular or reticulonodular disease

upper lobe prominent

32
Q

pulmonary langerhans histocytosis HRCT

A

small cysts in addition to nodular and reticulonodular changes
cysts may rupture and cause penumothorax
some cases extensive honeycombing

33
Q

pulmonary langerhans histocytosis course

A

natural history is variable
self limited-> extensive disease
no proven Tx
smoking cessation, corticosteroids

34
Q

lymphangioleiomyomatosis

A

rare pulmonary disease
characterized by proliferation of atypical smooth mm around vessels
women of childbearing age
tuberous sclerosis complex genetically predisposes

35
Q

lymphangioleiomyomatosis clinical

A

dyspnea and cough
vascular-> hemoptysis
lymph ostruction -> chylous pleural effusion
airway obstruction
rupture of cysts -> spontaneous pneumothorax

36
Q

lymphangioleiomyomatosis CXR

A

reticular pattern, cystic changes

lung volumes normal or increased

37
Q

lymphangioleiomyomatosis HRCT

A

cystic disease throughout parnechyma

38
Q

lymphangioleiomyomatosis PFT

A

obstructive, restrictive, both

39
Q

Goodpastures Tx

A 
palsmapheresis
immunosupression (prednisone)
40
Q

wagners

A

cANCA

URTI

41
Q

wagners Tx

A

cyclophosphamide

prednisone

42
Q

churg-strauss

A 
upper and lower respiratory tracts
preceded by allergic disorders
peripheral and lung eos
incrased IgE
rashes
43
Q

churg-strauss CXR

A

bilateral patchy, fleeting infiltrated
diffuse nodular infiltrates
difuse reticulonodular infiltrates

44
Q

churg-strauss biopsy

A

granulomatous angiitis or vasculitis

45
Q

churg-strauss Tx

A

corticosteroids

46
Q

chronic eosinophillic pneumonia

A

pulmonary interstitium and alveolar spaces infiltrated by eos and macros

47
Q

chronic eosinophillic pneumonia clinical

A

weeks-months
fever, weight loss, dyspnea, productive cough
pulmonary inflitrates w/peripheral distruction
increased esos in peripheral smear
BAL w/increased eos

48
Q

chronic eosinophillic pneumonia Tx

A

dramatic response to corticosteroids w/in days to weeks

prolonged for months to prevent recurrence

49
Q

pulmonary alveolar proteinosis

A

primary pathologic process affects the alveolar spaces, not alveolar cells
spaces filled w/proteinaceous phospholipid

50
Q

pulmonary alveolar proteinosis clinical

A 
dyspnea and cough
b/l alveolar infiltrates
HRCT crazy paving pattern due to thicking of interlobular septa
ground glass
superimposed nocardia infection
51
Q

pulmonary alveolar proteinosis Tx

A

whole-lung lavage

prognosis good

52
Q

hypersensitivity pneumonitis

A

hyperimmune respirartory syndrome

53
Q

hypersensitivity pneumonitis Tx

A

avoid causative agent

corticosteroids

54
Q

suspect hypersensitivty penumonitis

A

intermittent pulmonary and systemic symptoms
progressive pulmonary symptoms w/interstitial CXR changes
non-resolving pneumonia
agriculture and cattle farming
bird keeping
contaminated home
hot tubs

55
Q

acute hypersensitivity pneumonitis

A

abrupt onset of cough, dyspnea, fever, chest pain
symtoms 4-6 hours after exposure
subside w/in 24 hours

56
Q

subacute hypersensitivity pneumonitis

A

more gradual development

less severe

57
Q

chronic hypersensitivity pneumonitis

A

insidious progressive dyspnea, cough, weight loss, fatigue

most progress toward pulmonary fibrosis w/respiratory failure

58
Q

hypersensitivity pneumonitis CXR

A

acute- reticulonodular inflitrates

chronic- diffuse interstitial fibrosis

59
Q

hypersensitivity pneumonitis HRCT

A

ground glass opacities

chronic w/traction bronchietctasis, honeycombing, and fibrosis

60
Q

hypersensitivity pneumonitis PFTs

A

restriction
small lung volumes
decreased diffusion capactity

Resp II (15 decks)

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