Interstitial Lung Diseases

Front 1

Order of pathogenesis of diffuse ILD.

Back 1

injury -> alveolitis -> cellular and ct response -> repair or fibrosis

Front 2

Six agents of injury that can lead to diffuse ILD.

Back 2

1. ROS
2. serine proteases
3. cationic proteins
4. immune mechanisms
5. drugs
6. arachadonic acid metabolites

Front 3

A predominate Th2 type response, AKA humoral response, leads to _____.

Back 3

fibrosis

Front 4

A predominate Th1 type response, AKA cellular response, leads to _____.

Back 4

healing

Front 5

Lebow's Classification System consisted of two broad categories:

Back 5

1. Acute Interstitial Pneumonias - Diffuse Alveolar Damage
2. Chronic Interstitial Pneumonias - UIP, DIP, LIP, GIP

Front 6

The key to identifying UIP histologically?

Back 6

spatial and temporal heterogeneity

Front 7

Areas just underneath epithelial surface w/ fibroblastic proliferation, characteristic of UIP

Back 7

fibroblastic foci

Front 8

DIP is characterized by?

Back 8

accumulations of macrophages w/in alveolar space

Front 9

RBILD is only seen in?

Back 9

smokers, relatively mild respods well to smoking cessation

Front 10

Rapid course over typically a few weeks from onset to death.

Back 10

AIP

Front 11

The key difference in UIP vs. NSIP.

Back 11

temporal appearance: UIP is variegated (varied across section of lung) while NSIP has more uniform appearance (mostly inflammatory, or mostly fibrotic)

Front 12

____ is prominent in NSIP compared to UIP.

Back 12

Interstitial inflammation

Front 13

Honeycombing most frequently seen in ____

Back 13

UIP

Front 14

T/F: Occasionally you'll see organizing pneumonia in UIP but never in NSIP.

Back 14

False. Other way around.

Front 15

Hyaline membranes are occasionally seen in ____.

Back 15

AIP

Front 16

Most common symptoms of ILD?

Back 16

dyspnea and cough (nonproductive)

Front 17

Most important early sign in ILD?

Back 17

tachypnea

Front 18

Physical exam findings in ILD?

Back 18

digital clubbing, crackles or rails when listening to chest (Velcro)

Front 19

Velcro sounds in ILD heard during?

Back 19

mid-inspiration, compared to late inspiration heard w/ pulmonary edema

Front 20

Better prognosis: UIP or NSIP?

Back 20

NSIP

Front 21

UIP responds well/poorly to steroids?

Back 21

poorly

Front 22

DIP and NSIP respond well/poorly to steroids?

Back 22

well

Front 23

Three physiologic characteristics of restrictive lung disease:

Back 23

reduced lung volumes, reduced compliance, diffusion impairment

Front 24

People with ILD, what typically drops w/ excercise?

Back 24

O2 saturation

Front 25

Typically all lung volumes are _____ ______ in ILD.

Back 25

reduced proportionally

Front 26

On high resolution CT, abnormalities in ILD are more clustered and severe where?

Back 26

on the peripheral aspects of the lung

Front 27

Radiographic honeycombing is a sign of the ______ of ILD.

Back 27

end stages

Front 28

Two main clinical classifications of ILD:

Back 28

Known and Unknown etioligies

Front 29

Name some of the known etiologies of ILD:

Back 29

silica, gases/fumes, pigeon droppings, drugs, radiation

Front 30

Most cases of ILD are of ____ etiology.

Back 30

unknown

Front 31

Causes of unknown etiology ILD.

Back 31

UIP, collagen vascular disease like lupus, eosinophilic pneumonias and Wegener's granulomatosis

Front 32

Possible therapy for ILD?

Back 32

antiinflammatories (steroids?), azothioprine, colchicine (adjunct), N-acetyl cysteine

Front 33

Last ditch effort for patients w/ ILD?

Back 33

lung transplant

Front 34

Prognosis of UIP

Back 34

50% mortality in 5 years