Interstitial lung disease

Question 1

characterization of ILD

Answer 1

cellular infiltration
scarring
architectural disruption of parenchyma

Question 2

ILD can compromise what locations of the lung?

Answer 2

alveolar wall
septum interstitium
lumina and walls of small airways

Question 3

clinical presentation of ILD

Answer 3

acute, subacute or chronic 
progressive dyspnea on exertion 
dry cough 
restrictive PFTs 
CXR / CT - interstitial infiltrates with ground glass opacities

Question 4

physical exam for ILD

Answer 4

clubbing 
erythema / nodosum / arthritis / rash 
tachypnea 
dry crackles / squeaks 
increased right heart pressures

Question 5

what may be the earliest change in PFTs in the context of ILD?

Answer 5

decreased DLCO

Question 6

what is the radiographic distribution of ILD?

Answer 6

upper / lower

central / peripheral

Question 7

what is the granulomatous ILD?

Answer 7

sarcoidosis

Question 8

which idiopathic interstitial pneumonitis (IIP) has the worst prognosis?

Answer 8

idiopathic pulmonary fibrosis (IPF)

Question 9

what are the radiographic characteristics of IPF?

Answer 9

1. lower lobes and peripheral distribution of reticular infiltrates
2. fibrotic changes

Question 10

what is the clinical use for nintedanib?

Answer 10

IPF

Question 11

what are two medications for IPF?

Answer 11

nintedanib

pirfenidone

Question 12

what is the clinical use for pirfenidone?

Answer 12

IPF

Question 13

what is the radiographic presentation of nonspecific interstitial pneumonitis (NSIP)?

Answer 13

ground glass opacity symmetrically in the lower lung zones, with or without reticular infiltrates

Question 14

DIP

Answer 14

desquamative interstitial pneumonitis

Question 15

RB-ILD

Answer 15

respiratory bronchiolitis associated ILD

Question 16

DIP and RB-ILD are associated with what activity?

Answer 16

smoking

Question 17

AIP

Answer 17

acute interstitial pneumonitis

Question 18

DAD

Answer 18

diffuse alveolar damage

Question 19

what is the radiographic presentation of AIP / DAD?

Answer 19

diffuse, bilateral air space and reticular infiltrates

Question 20

what is the problem associated with AIP / DAD? what is the treatment?

Answer 20

oygenation

supportive

Question 21

COP

Answer 21

cryptogenic organizing pneumonia

Question 22

patients with COP present with what type of symptoms?

Answer 22

pneumonia

Question 23

what is the treatment for COP? what is the prognosis?

Answer 23

corticosteroids

good

Question 24

what is the radiographic presentation for COP?

Answer 24

patchy infiltrates, unilateral or bilateral
50% small nodular opacities
consolidation and air bronchogram seen on HRCT
subpleural and peribronchiolar distribution

Question 25

what is the mechanism for hypersensitivity pneumonitis?

Answer 25

cell mediated immunity - activation of alveolar macrophages and CD8 influx with ill defined granuloma formation

Question 26

what is the acute presentation of hypersensitivity pneumonitis? (onset, PE, imaging)

Answer 26

abrupt onset of flu like symptoms tachypnea and diffuse fine crackles radiograph - normal or patchy, micronodular opacities

Question 27

how does chronic presentation of hypersensitivity pneumonitis differ from acute?

Answer 27

digital clubbing | irreversible respiratory findings - FIBROSIS

Question 28

what drug can cause commonly cause drug induced ILD?

Answer 28

amiodarone

Question 29

what are highly suggestive radiographic findings for HP (hypersensitivity pneumonitis)?

Answer 29

ground glass opacification and centrilobular nodules

Question 30

what are the radiographic findings of amiodarone toxicity?

Answer 30

ground glass and reticular opacities increased attenuation liver and spleen seen on abdominal cuts

Question 31

what are the two presentations for nitrofurantoin toxicity? what are the symptoms?

Answer 31

acute and chronic onset acute - peripheral blood eosinophilia chronic - increased serum Ig, serum transaminases, ESR, ANA+

Question 32

what is the radiographic presentation of acute eosinophilic pnuemonia?

Answer 32

bilateral patchy ground glass and/or reticular opacities | increased eosinophilic count (over 25%) - key

Question 33

what is the radiographic presentation of chronic eosinophilic pnuemonia?

Answer 33

bilateral peripheral or pleural based opacities described as "photographic negative" of pulmonary edema subpleural consolidations, predominantly upper lobes

Question 34

what are the features of lymphangioleiomyomatosis?

Answer 34

``` progressive cystic lung disease women of childbearing age associated with tuberous sclerosis proliferation of SMCs dyspnea on exertion, cough, hemoptysis ```

Question 35

what is the radiographic presentation of lymphangioleiomyomatosis?

Answer 35

diffusely distributed thin walled cysts

Question 36

what is the evaluation of ILD following history and PE?

Answer 36

chest radiography blood tests PFTs