Diffuse lung disease Flashcards

1
Q

IPF: mean survival, prognosis, symptoms, patient population

A

most common idiopathic interstitial pneumonia; second worse prognosis (2-4 yrs survival)

dry cough, >50 yo

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2
Q

pathological diagnosis of IPF

A

usual interstitial pneumonia

interstitial fibroblastic foci and chronic alveolar inflammation

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3
Q

usual interstitial pneumonia causes

A

IPF, collagen vascular disease, drug injury, asbestosis

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4
Q

UIP imaging

A

early: irregular reticulation in posterior subpleural lung bases
late: fibrosis, traction bronchiectasis, posterior subpleural honeycombing

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5
Q

nonspecific interstitial pneumonitis: patient population, prognosis, forms

A

younger patients 40-50yo

responds to steroids

fibroitic and cellular NSIP forms

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6
Q

causes of NSIP

A

idiopathic, most common manifestation of collagen vascular disease, drug reaction, occupational exposure, dermatomyositis

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7
Q

fibroitic NSIP

A

GCO with fine reticulation and traction bronchiectasis

lacks honeycombing (honeycombing with UIP)

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8
Q

cellular NSIP

A

GCO without significant fibroitic changes; worse prognosis than fibrotic NSIP

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9
Q

NSIP imaging

A

sparing of immediate subpleural lung (unlike UIP)

posterior peripheral lower lobes

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10
Q

cryptogenic organizing pneumonia, previously bonchiolitis obliterans organizing pneumonia (BOOP): prognosis, treatment

A

good prognosis, may resolve completely with steroids

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11
Q

pathology of COP

A

granulation tissue polyps that fill airways in response to infection, drug inhalation, inhalation

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12
Q

COP imaging

A

mixed consolidation and ground glass opacities in peripheral and peribronchovasculat distribution

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13
Q

reverse halo/atoll sign

A

specific for OP

central lucency surounded by ground glass halo

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14
Q

halo sign

A

invasive aspergilus

central opacity with ground glass opacity

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15
Q

respiratory bronchiolitis-interstitial lung disease (RB–ILD)

A

common in smokers; pigmented macrophages present in respiratory bronchioles

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16
Q

histology respiratory bronchiolitis- ILD

A

sheets of macrophages filling terminal airways; sparing of alveoli

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17
Q

RB ILD imaging features

A

centrilobular nodules, patchy ground glass opacities

random distribution

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18
Q

desquamative interstitial pneumonia imaging

A

diffuse basal predominant patchy/subpleural ground glass opacification; few csts

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19
Q

DIP histology

A

brown pigmented macrophages; sheets of macrophages extend into alveoli

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20
Q

lymphoid interstitial pneumonia

A

rare; associated with Sjogren syndrome or HIV

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21
Q

histology lymphoid interstitial pneumonia

A

diffuse infiltration of interstitium by lymphocytes; distortion of alveoli

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22
Q

imaging lymphoid interstitial pneumonia

A

diffuse/lower lobe predominant ground glass; thin walled perivascular cysts present

may cause pneumothorax in advanced cases

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23
Q

acute interstitial pneumonia aka diffuse alveolar damage

A

seen with ARDS; acute onset and worst prognosis

caused by surfactant destruction

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24
Q

phases of acute interstitial pneumonia

A

early: exudative; hyaline membranes, diffuse alveolar infiltration by immune cells; noncardiogenic pulmonary edema

chronic: organizazing: alveolar wall thickkening due to granulation tissue; >1 w after injury

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25
AIP imaging
extensive geographic GGO
26
inhalation lung disease localization
upper lobes lower lobes have better blood flow/lymphatic drainage
27
hypersensitivity pneumonitis (HSP)
hypersensitivity reaction causes: bird proteins, thermophilic actinomycetes; organic dust
28
phases of HSP
acute: inflammatory exudate fill alveoli; GGO/consolidations; small centrilobular nodules subacute: centrilobular GG nodules; mosaic attenuation; "head cheese sign" chronic: upper lobe predominant pulmonary fibrosis
29
pneumoconioses
inorganic dust inhalation (silicosis, coal workers pneumoconiosis, asbestosis)
30
most common types of pneumoconioses
silicosis/CWP
31
imagine findings of silicosis/coal workers lung
uncomplicated: multiple upper lobe predminant centrilobular/subpleural nodules complicated: large conglomerate masses/massive fibrosis
32
eggshell lymph node calcifications
silicosis, less common CWP
33
increased risk of ? with pneumoconiosis
TB
34
Caplan syndrome
rheumatoid arthritis + CWP or silicosis necrobiotic rheumatoid nodules superimposed on centrilobular/subpleural nodules of pneumoconiosis
35
asbestosis, distribution
inhalation of asbestosis fibers > pulmonary fibrosis > UIP pathology primarily affects lower lobes since they are too large to be removed by alveolar macrophages/lymphatics
36
imaging findings of asbestosis
similar to IPF but have pleural thickening and plaques
37
eosinophilic lung disease
simple pulmonary eosinophilia/Loffler syndrome; chronic eosinophilic pneumonia
38
simple pulmonary eosinophilia/Loffler syndrome
transient, migratory areas of focal consolidation; elevated eosinophil count in peripheral smear similar appearance with parasitic disease and drug reactions
39
chronic eosinophilic pneumonia
extensive alveolar filling/interstitial infiltration with inflammatory eosinophils patchy, peripheral, upper lobe predominance responds to steroids
40
pulmonary vasculitis
churg straus/allergic angiitis and granulomatosis, microscopic polyangitis, Wegner granulomatosis
41
Churg Strauss/Allergic Angiitis and Granulomatosis
small vessel vasculitis + asthma + peripheral eosinophilia P-ANCA+ peripheral consolidation/ground glass
42
Microscopic polyangitis
common cause of pulmonary hemorrhage with renal failure P-ANCA + diffuse central-predominant ground glass representing hemorrhage
43
Wegener granulomatosis
systemic small vessel vasculitis with sinusitis + lungs + renal C-ANCA+ can cause airway stenosis (nasopharyngeal/eustachian tube obstruction) multiple cavitary lung nodules that don't respond to antibiotics
44
pulmonary manifestations of drug toxicity
pulmonary edema, ARDS, organizing pneumonia, eosinophilic pneumonia, bronchiolitis obliterans, pulmonary hemorrhage, NSIP, UIP
45
radiation lung injury
confined to radiation port; crosses anatomic margins radiation pneumonitis: 1-4 months radiation fibrosis: 6-12 mo
46
sarcoidosis
noncaseating granulomas that form nodules/masses throughout body pulmonary sarcoid > fibrosis mid/upper lung predominance
47
CXR staging of sarcoidosis
Stage 0: Normal radiograph. Stage 1: Hilar or mediastinal adenopathy only, without lung changes. Stage 2: Adenopathy with lung changes. Stage 3: Diffuse lung disease without adenopathy. Stage 4: End-stage fibrosis.
48
lateral radiograph "donut sign"
adenopathy circumferentially encircling trachea
49
CT findings of sarcoid
adenopathy, upper lobe predominant perilymphatic nodules of varying sizes/granulomas bronchial involvement > air trapping galaxy sign with coalescing nodules into mass
50
pulmonary langerhans cell histiocytosis
smoking related lung disease nodules associated with airways that form irregular cysts; upper lobe predominant, peribronchovascular nodules spare costophrenic sulci
51
smoking related lung disease can cause spontaneous pneumothorax may also affect bones, DI from hypophysitis, skin involvement
pulmonary langerhans cell histiocytosis, RB-ILD, DIP
52
DDX for pulmonary and bone involvement
pulmonary langerhans cell histiocytosis, malignancy, TB, fungal disease, sarcoid, Gaucher
53
PLCH progression
nodules > cavitary nodules > IRREGULAR cysts
54
treatment for PLCH
steroids | smoking cessation
55
pulmonary alveolar proteinosis (PAP)
alveoli filled with proteinaceous lipid-rich material
56
imaging of PAP
XR: pulmonary edema, perihilar opacification CT: cazy paving in areas of geometric ground glass
57
ddx for crazy paving
pulmonary alveolar proteinosis, pneumocystis pneumonia, COP, bronchoalveolar carcinoma, lipoid pneumonia
58
PAP can have superimposed infection with what organism?
Nocardia
59
treatment for PAP
bronchoalveolar lavage
60
lymphangioleiomyomatosis
diffuse cystic lung disease cauesed by bronchiolar obstruction and lung destruction women of child bearing age can have pneumothorax and chylous leural effusion
61
tuberous sclerosis triad
seizure, mental retardation, adenoma sebaceum
62
LAM and TS
both can have identical appearing lung disease
63
imaging apperance of LAM
numerous thin walled lung cysts; round and regular cysts (unlike LCH) can affect all lobes (LCH is upper lobe predominant)