Ucsf: Video H 69 Part 1 Diffuse Airway Disease (Interstial Disease)

Front 1

What is the does interlobular thickening look like

Back 1

reticular

Front 2

What is interlobular thickening

Back 2

thickening of the septa that surround the secondary pulmonary lobules

Front 3

What is the approximate size of a secondary lobule

Back 3

2cm

Front 4

What is in the center of a secondary lobule

Back 4

centrilobular arterty

Front 5

What can you see in the septa occasionally

Back 5

pulmonary vein branches

Front 6

Does pulmonary edema cause interlobular septal thickening

Back 6

yes

Front 7

Should you ignore IST if it is not the predominate finding

Back 7

yes, almost everyone has some IST

Front 8

What are the common causes of IST

Back 8

interstitial pulmonary edema
lymphangitis spread of neoplasm
fibrosis
any other interstitial infiltration

Front 9

What is the cause of kerley b lines

Back 9

interlobular septal thickening

Front 10

When do you expect to see kerley b lines

Back 10

pulm edema

Front 11

What is the plain film equivalent of IST

Back 11

ikerley b lines

Front 12

What should you think of with unilateral IST

Back 12

lymphangitic spread of cancer

Front 13

What does honeycombing look like

Back 13

subpleural cystic change

Front 14

What are the causes of most of the interstial pneumonias

4

Back 14

idiopathic (IPF, and all the idiopathic interstial pneumonias)
collagen vascular disease (scleroderma, SLE, RA, dermatocytisis)
drugs (amiodarone and tons of others)
inhalation (hypersensitivity pneumonitis which is organic and inhalation of inorganic substance)

Front 15

What are 'idiopathic' interstial pna histologic classifications
7

Back 15

UIP (usual interstial pna)
NSIP (non-specific interstial pna)
OP (organizing pna)
DIP (desquamative interstial pna)
RB-ILD (respiratory bronchiolitis interstial lung disease)
LIP (lymphoid interstial pna)
AIP (acute interstial pna)

Note idiopathic is a misnomer because although these histologic patterns can be caused by idiopathic disease there are also known causes which will be shown on later flashcards)

Front 16

What are two types of organizing pneumonia

Back 16

BOOP (bronchiolitis obliterans with organizing pna)
COP (cryptogenic organizing pneumonia)

Front 17

What idiopathic clinical syndrome is histological pattern of UIP associated with

Back 17

IPF

Front 18

What are other non-idiopathic causes the histologic pattern UIP (besides ipf)
3

Back 18

CVD (scleroderma, RA, SLE, dermatomyositis) , asbestosis, silicosis, drug fibrosis, end stage hypersensitiivty pneumonitis (organic substances NOT silicosis and asbestosis)

Front 19

What idiopathic clinical syndrome causes the histologic pattern NSIP

Back 19

idiopathic NSIP

Front 20

What are other non-idiopathic causes the histologic pattern NSIP (besides idiopathic NSIP)
2

Back 20

CVD (sleraderma, RA,dermatomyositis, SLE)
Drugs

Front 21

What idiopathic clinical syndrome causes the histologic pattern OP

Back 21

COP or idiopathic BOOP

Front 22

What non-idiopathic clinical entities cause the histologic pattern of OP (in addition to idiopathic COP and BOOP)

5

Back 22

Infection
CVD (scleraderma, SLE, RA, dermatomyositis)
Drugs
fumes
HP

Front 23

What idiopathic clinical syndrome causes the histologic pattern DIP

Back 23

idiopathic DIP

Front 24

What are other non-idiopathic causes the histologic pattern of DIP
2

Back 24

smoking and fumes

Front 25

What does COP stand for

Back 25

cryptogenic organizing pna

Front 26

Can UIP, NSIP, OP and DIP be the result of non-idiopathic diseases?

Back 26

Yes

Front 27

Can 'idiopathic' interstial pna have different patterns

Back 27

several patterns (which are the different histologic subtypes)

Front 28

Is usual interstial pna common

Back 28

yes, hence the name usual

Front 29

Describe the HRCT findings of UIP

Back 29

reticulations, traction bronchiectasis, honeycombing, and associated ground glass opacification

Front 30

What percent of UIP have honeycombing

Back 30

70

Front 31

Where is the predominance of UIP in the lung

Back 31

basal posterior lower lobe predominence

Front 32

What another name for idiopathic UIP

Back 32

idiopathic pulmonary fibrosis

Front 33

What is the prognosis of UIP and IPF

Back 33

poor

Front 34

What are additional causes of the histologic pattern UIP

Back 34

CVD (scleroderma, RA, SLE, dermatomyositis) , asbestosis, silicosis, drug fibrosis, end stage hypersensitiivty pneumonitis (organic substances NOT silicosis and asbestosis)

Front 35

What are the size of the honeycombs on CT

Back 35

3-10mm

Front 36

Does honeycombing occur subpleural

Back 36

yes

Front 37

How does the cystic lucencies progress in honeycombing

Back 37

early isolated cyst then eventually several layers and will share walls

Front 38

On plain film why are the lung volumes reduced with UIP

Back 38

fibrosis and lung restriction

Front 39

What is the typical finding of UIP

Back 39

reticular pattern in lower lung fields and well as low lung volumes

Front 40

What is the earliest abnormality of UIP on plain film

Back 40

reticular pattern in the posterior costophrenic angle on lateral film

Front 41

What is the differential diagnosis of honeycombing

3

Back 41

1-UIP and its cause: IPF, CVD (RA, scleroderma, sle, dermatomyositis), drugs, inhalation (chronic hypersensitivity, silicosis,asbestosis)
2-end stage sarcosis
3-NSIP

Front 42

What is the mcc of UIP

Back 42

IPF

Front 43

What are the patients age who develop IPF

Back 43

typically greater than 50

Front 44

What is the histologic pattern with IPF

Back 44

UIP

Front 45

What is traction bronchiectasis

Back 45

the airways are dilated in an odd corkscrew fashion

Front 46

When do yo typically see the irregular reticulatons with IPF

Back 46

early stages before honeycombing

Front 47

Does basal honeycombing on HRCT have a high predictive value for UIP

Back 47

yes

Front 48

In the absence of CVD, drugs, asbestosis, organic antigens what is the most likely diagnosis

Back 48

IPF

Front 49

Is lung biopsy performed in IPF

Back 49

no

Front 50

35 year old smoker with rhematoid arthritis and honeycombing is

Back 50

has rheumatoid lung disease (which has the UIP histology)

Front 51

A patient with honey combing and pleural plaques

Back 51

asbestosis (which has a UIP histologic pattern)

Front 52

Why is it important not just diagnose honeycombing as IPF

What is it called when lungs have pathology from organic material

Back 52

there are differences in prognosis
for example IPF is fatal within 5 years while CVD caused UIP the patients will live much much longer.

hypersensitivity pneumonconisos
iorganic is not hp

Front 53

What is more common UIP or NSIP

Back 53

UIP

Front 54

What is a major radiographic and histologic differentiation of UIP and NSIP

Back 54

UIP has hetergeneous inflammation while NSIP is homogeneous

Front 55

What are the two forms of NSIP

Back 55

inflammatory or fibrotic or combination

Front 56

Is NSIP a common pattern in CVD

Back 56

yes

Front 57

What is the prognosis of NSIP

Back 57

good response to treatment

survival is 80-90%

Front 58

What are two typical patterns seen in NSIP on HRCT

Back 58

GGO and reticular opacities

Front 59

What does GGO alone mean

Back 59

just inflammatory and no fibrosis yet

Front 60

What does it mean if there is reticulation and GGO

Back 60

cellular or fibrotic

Front 61

What does traction bronchiectasis or honeycombing in the setting of NSIP mean

Back 61

likely fibrotic

Front 62

Is honeycomming common in NSIP

Back 62

no (1-5%)

Front 63

Where is the common location of NSIP on HRCT?

Back 63

lower lobe posterior peripheral predominance

Front 64

What is spared in 20-50% of NSIP

Back 64

subpleural lung

Front 65

NSIP

Back 65

non-specific interestial pna

Front 66

Can NSIP have a lot of honeycombing

Back 66

no,if any (1-5%)

Front 67

What is classic for NSIP?

Back 67

sparing of the subpleural lung

Front 68

What is better survival UIP or NSIP?

Back 68

NSIP (both the fibrotic and inflammatory form)

Front 69

What is another name for inflammatory NSIP

Back 69

cellular nsip

Front 70

When is the histologic pattern of Respirotory bronchiolitis seen?

Back 70

smokers

Front 71

What is RB plus symptoms called

Back 71

RB Iterstial lung disease

RB-ILD

Front 72

Is DIP similar to RB

Back 72

yes, it is similar histology but more extensive

Front 73

What is more common UIP, NSIP or DIP

Back 73

UIP and NSIP are much more common than DIP

Front 74

What percent of smokers who have lung biopsies will have RB

Back 74

almost 100% but they usually dont have symptoms

Front 75

What is the prognosis of DIP

Back 75

very good it is treated with steroids

Front 76

What is seen histologicaly with DIP

Back 76

intraalveolar macrophages

Front 77

Is there alot of fibrosis with DIP

Back 77

no

Front 78

What is the main finding on HRCT in a pt with RB-ILD

Back 78

localized and often centrilobular inflammation accentuated a small amount of GGO

Front 79

What is the spectrum of RB, RB-ILD and DIP

Back 79

1st - RB (100% on biopsy of asymtomatic smokers)
2nd- RB-ILD (RB pattern on biopsy but has symptoms)
3rd- DIP (more extensive histologically causing GGO)

Front 80

What is seen on HRCT in a patient with DIP

Back 80

GGO that is patchy and subpleural in distribution and more extensive than RB-ILD