ILD

Question 1

what are the physiologic properties of ILD?

Answer 1

• restriction - decreased compliance - abn gas exchange

Question 2

in idiopathic pulmonary fibrosis what is issue?

Answer 2

the initial inflammation is unknown but TGF-beta is involved by activating fribroblasts leading to deposition of collagen thus fibrosis

Question 3

what do see in PFTs in ILD?

Answer 3

-decreased TLV, FRC and RV - decreased FEV1, FVC and either normal or increased FEV1/FVC - decreased DLCO

Question 4

what is the course of IPF?

Answer 4

it is pretty grim, not high survival rates however there is new tx. that slows down the decline in FEV1 and thus might be able to improve survival rates but thatis unknown

Question 5

when you one use glucocorticoids in treating certain ILD?

Answer 5

if there are inflammatory (eosinophils, lymphocytes or granulomatous) or ground glass/alv infiltrates (sarcoidosis, COP, ceP) are present

Question 6

what is there is extensive honeycombing?

Answer 6

no effective treatment, one should ocnsifer lung transplant

Question 7

what do both ninetdanib and pirfenidone do?

Answer 7

slows down decline in function and increases exercise tolerance

Question 8

inorganic dusts

Answer 8

pneumoconiosis

Question 9

organic proteins

Answer 9

hypersensitivity pneumonitis

Question 10

hallmark of silicosis

Answer 10

ineffective clearance

Question 11

hallmark of HP

Answer 11

exaggerated immune response

Question 12

amphiboles

Answer 12

asbestos fiber

Question 13

worse asbestosis manifestation in pleura

Answer 13

mesothelioma

Question 14

treatment of HP

Answer 14

removal of the causative agent, and steroids in symptomatic patients

Question 15

silo filler’s lung and metal fume fever

Answer 15

are direct tissue injury

Question 16

byssinosis

Answer 16

inhalation of unprocessed cotton dust causing direct airway tissue injury

Question 17

RADS

Answer 17

irritant induced asthma where caustic fumes cause direct airway damage

Question 18

when do we start to see asthma like symtpoms in RADS?

Answer 18

within 12 hr

Question 19

what should we do about RADS?

Answer 19

treat it like asthma. most improve over time but may lead to permanent airway hyperresponsiveness

Question 20

in immunologic occupational asthma we see ____ and we should expect what are two of the most common cause?

Answer 20

we see IgE mediated response and there is latency between exposure and symptoms - western red cedar and baker’s asthma

Question 21

what can we do to determine if it is occupational asthma?

Answer 21

PEFR

Question 22

treatment of occupational asthma

Answer 22

remove exposure, bronchodilators and anti-inflammatory (steroids)

Question 23

true or false asbestosis can be seen quickly?

Answer 23

nope disease seen in many years after exposure but one can see pleural plaques with recent exposure

Question 24

where are the silicosis particles mainly deposited? who are the main instigators? where can nodules be seen in?

Answer 24

deposited in upper lobes - macrophages release pro-inflammatory and pro-fibrotic mediators - seen in hilar lymph nodes

Question 25

HP examples

Answer 25

- farmer's lung - maple bark disease - bird-fancier's lung -humidifier or air conditioner lung

Question 26

HP hallmark in histology

Answer 26

lymphocytes

Question 27

Inflammatory pattern characterized by the presence of collections of epithelioid histiocytes (macrophages), with or without multinucleated giant cells (these are also histiocytes) and necrosis

Answer 27

granuloma granulomatous

Question 28

prototypical idipathic granulomatous disorder

Answer 28

sarcoidosis

Question 29

sarcoidosis hallmark

Answer 29

non-necrotizing granulomas in different organs and increased serum ACE

Question 30

Vasculitis and necrotizing granulomas in upper and lower respiratory tract and necrotizing or crescentic glomerulonephritis

Answer 30

GRANULOMATOSIS WITH POLYANGIITIS

Question 31

GRANULOMATOSIS WITH POLYANGIItis has a c-ANCA

Answer 31

positive test

Question 32

LANGERHANS CELL HISTIOCYTOSIS

Answer 32

proliferation of langerhan cells/APC and strongly associated with smoking

Question 33

Organizing pneumonia histologic pattern without known cause

Answer 33

cryptogenic organizing pneumonia

Question 34

dissuse alveolar damage

Answer 34

acute interstitial pneumonia

Question 35

alveolar macrophages sfill alveolar spaces leading to mild interstitial fibrosis but there is good response to smoking ceddation and steroids

Answer 35

desquamative interstitial pneumonia

Question 36

You are evaluating a patient with dyspnea, non-productive cough, and inspiratory crackles on exam. A chest CT shows diffuse ground glass opacities. Which of the following would NOT be expected? - decreased FEV1, decreasedFVC, nl FEV/FVC, decreasedTLC, decreasedDLCO - decreased compliance -Fibrosis on lung biopsy -Rapid shallow breathing - H/O tobacco smoking

Answer 36

-Fibrosis on lung bio-Fibrosis on lung biopsypsy

Question 37

Do we see hypercapnia in ILD?

Answer 37

yea, they have a rapid shallow breathing, cystic airspaces and diffusion abnormality at later stages

Question 38

Answer 38

asbestos (ferruginous) body

Question 39

Answer 39

Birbeck granules are seen ultrastructurally within langerhans

Question 40

Answer 40

birefringent silica particles under polarized ligh

Question 41

Answer 41

Diffuse airspace filling by macrophages with light brown cytoplasmic pigment; In RB-ILD, the process is less extensive and has bronchiolocentric distribution

Question 42

Answer 42

geographic necrosis

Question 43

Answer 43

granulomatosis with polyangiitis the lung shows multiple cavitating mass-like lesions

Question 44

Answer 44

HP- airway centered lymphocyte predominant inflammation extending into alveolar septa

Question 45

Answer 45

interstitium infiltrated by lymphocytes

Question 46

Answer 46

interstitium widened by fribrosis

Question 47

Answer 47

LHC- Langerhans have a prominent nuclear grooves

Question 48

Answer 48

microscopic cysts-honeycombing

Question 49

Answer 49

normal interstitium

Question 50

Answer 50

normal interstitium with elastic fibers

Question 51

Answer 51

pathcy fibrosis- abestosis

Question 52

Answer 52

sarcoidosis- notice the little background inflammation

Question 53

Answer 53

sarcoidosis- packed histiocytes

Question 54

Answer 54

silicotic nodule

Question 55

what is this?and what do they suggest?

Answer 55

what is this- diffuse reticular infiltrates and what do they suggest- that fibrosis is presen

Question 56

## Footnote what is this? and what do they suggest?

Answer 56

## Footnote what is this- ground glass opacities and what do they suggest- infection,blood and inflammation