Lec 18 Pathology of Interstitial Lung Disease

Question 1

What is honeycomb lung

Answer 1

end stage lung state = final result of many interstitial lung disease

have severe fibrosis with architectural remodeling resulting in large airspaces with thick fibrous walls

Question 2

What happens to compliance in interstitial lung disease?

Answer 2

decreased compliance

Question 3

What happens to lung volumes in interstitial lung disease?

Answer 3

decreased lung volumes

Question 4

What happens to pulmonary BP in interstitial lung disease?

Answer 4

get pulmonary htn

Question 5

How does fibrosis happen in interstitial lung disease?

Answer 5

• have abnormal repair w/ replacement by dense connective tissue; loss of normal architecture; organ dysfunction

Question 6

Who gets usually interstitial pneumonia?

Answer 6

people > age 50

50% idiopathic
otherwise due to: collagen vascular disease or autoimmune disease; drug rxn

Question 7

What is clinical picture of usual interstitial pneumonia [UIP]?

Answer 7

exertional dyspnea, chronic evolution; secondary pulmonary HTN, cor pulmonale, cardiac failure

Question 8

What defines idiopathic pulmonary fibrosis?

Answer 8

UIP pattern on histology PLUS idiopathic disease clinically

Question 9

What do you see on histology with UIP?

Answer 9

patchy fibrosis; most pronounced beneath pleura and next to interlobular septa

fibrosis exhibits temporal heterogeneity = have fibroblast focus w/ blue/grey new fibrosis adjacent to dense collagenous scar of old fibrosis

Question 10

What is clinical course/treatment of usual interstitial fibrosis?

Answer 10

treat = steroid have minimal benefit; transplant in some pts

mean survival 3 yrs or less

Question 11

What is non-specific interstitial pneumonia?

Answer 11

similar presentation as UIP but younger pts; less severe clinical course

Question 12

Better prognosis for fibrosing or cellular subtype of non-specific interstitial pneumonia?

Answer 12

better prognosis for cellular; has biggest response to steroids

Question 13

What do you see on histology in cellular pattern of NSIP?

Answer 13

diffuse chronic inflammatory cell infiltrates
no fibrosis
no significant alveolar expansion

Question 14

What do you see on histology in fibrosing pattern of NSIP?

Answer 14

diffuse interstitial fibrosis w/ uniform appearance

fibrosis all same age = temporally uniform

Question 15

Who gets lymphocytic interstitial pneumonia [LIP]?

Answer 15

seen in pts with sjogren’s, HIV

Question 16

What do you see in lymphocytic interstitial pneumonia [LIP]?

Answer 16

small mature lymphocytes + infiltrate in alveolar septa = expanding the alveolar septa

radiologically = show diffuse ground glass changes w/ cysts

Question 17

What is clinical presentation of LIP?

Answer 17

presents as cough/dyspnea

Question 18

What is sarcoidosis? How does it classically present?

Answer 18

• multisystemic disease of unknown origin

- classically –> interstitial/nodular lung infiltrates and bilateral hilar adenopathy

Question 19

Who usually gets sarcoidosis?

Answer 19

20-40 y/o
F > M
90% black; rare in chinese, SE Asian

Question 20

What is prognosis of sarcoidosis?

Answer 20

65% recover
20% permanent pulm loss
small percent die of pulm fibrosis

Question 21

What are multi-system symptoms of sarcoidosis?

Answer 21

lungs: no gross lesions or small nodules

lymph nodes: hilar /mediastinal lymph nodes involved

liver/spleen involved in 75% cases

skin involved in 30-50%

eyes in 20-50%

Question 22

What is morphology of sarcoidosis?

Answer 22

non-necrotizing epithelioid granulomas usually tightly packed

in interstitium adjacent to bronchioles

Question 23

What is classic triad of hypersensitivity pneumonitis?

Answer 23

• chronic bronchiolitis/interstitial pneumonia
• poorly formed granulomas
• organizing pneumonia

Question 24

What is respiratory bronchiolitis?

Answer 24

accumulation of macrophages w/ fine granular brown pigment [smokers macrophages] in small airways adjacent to alveolar septa

may have mild chronic inflammation and fibrosis

Question 25

What is resp bronchiolitis associated interstitial lung disease?

Answer 25

speciic clinical situation in which patient is clincally thought to have interstitial lung disease and has RB as exclusive finding

Question 26

What is desquamative interstitial pneumonia [DIP]? clinical signs?

Answer 26

more diffuse than RB-ILD and more severe

insidious onset SOB

Question 27

Who gets desquamative interstitial pneumonia?

Answer 27

disease of current or past cigarette smokers

Question 28

What do you see on xray in desquamative interstitial pneumonia [DIP]?

Answer 28

bilateral lower lobe involvement

ground glass infiltrates

Question 29

What is treatment for desquamative interstitial pneumonia [DIP]?

Answer 29

steroids, smoking cessation

Question 30

What do you see microscopically in DIP?

Answer 30

diffuse intraalveolar macrophages w/ fine granular brown pigment

minimal changes in alveolar septa
no significant interstitial fibrosis

Question 31

What is langerhands cell histiocytosis?

Answer 31

bronchiolocentric fibrosis w/ stellate scar formation

variable number of langerhans

Question 32

Who gets langerhans cell histiocytosis?

Answer 32

male smokers

Question 33

Birbeck granule is a sign of what?

Answer 33

tennis raque shaped intracellular structure = characterstic of langerhans cell

Question 34

What is a ferruginous body?

Answer 34

inorganic substance w/ coating of iron and protein

can be graphite, ceramic, iron, etc

Question 35

What is an asbestos body?

Answer 35

ferruginous body formed on an asbestos fiber = characterized by clear internal core and beaded ferruginous coating

Question 36

What are manifestations of asbestos disease?

Answer 36

• pleural fibrosis and/ord pleural plaques

- pulmonary parenchymal fibrosis [asbestosis] –> end stage fibrosis + honeycomb lung

Question 37

What are pleural plaques?

Answer 37

well circumscribed plaques of dense collagen on parietal pleura and dome of diaphragm

do not contain asbestos bodies

Question 38

What is asbestosis?

Answer 38

diffuse interstitial fibrosis secondary to asbestos exposure

initial injury at bifurcation of small airways and ducts

fibrosis begins around resp bronchioles/alveolar ducts and extends distally

Question 39

Who is at risk for asbestosis?

Answer 39

shipyard worker, insulator, pipefitter

Question 40

Where does asbestosis begin?

Answer 40

in lower lobes subpleurally

begins around resp bronchioles/alveolar ducts ands extends distally

Question 41

What do you see in silicosis?

Answer 41

• marked silicotic nodules that enlarge and obstruct airways and blood vessels

Question 42

Who is at risk for silicosis?

Answer 42

miners, glass manufacturers, sandblasters, stone cutters

Question 43

What do you see grossly in silicosis?

Answer 43

early tiny discrete nodules in upper zones of lung –> progress to collagenous scards

nodules have stellate shape at edge

may have hilar node eggshell calcification

Question 44

What do you see in silicosis on microscopy in early vs late?

Answer 44

early = small nodules of fibroblasts and histocytes w/ cilia –> less cellular and more lyanizied w/ time

later = hyalinized and condensed collagen; needle-like spicules w/ pointed ned

Question 45

What do you see in simple coal workers pneumoconiosis?

Answer 45

cola macules + coal nodules scattered through lung; more in upper lobe and upper lower lobe near resp bronchioles

Question 46

What do you see in progressive massive fibrosis [complicated coal workers pneumoconiosis]?

Answer 46

intensely blackened scars; multiple containing dense collagen and pigment
center of lesion necrotic due to ischemia

Question 47

What is caplan syndrome?

Answer 47

rheumatoid arthritis and pneumoconiosis cause rapidly developing nodular pulm lesions histologically identical to rheumatoid nodules