Lec 18 Pathology of Interstitial Lung Disease Flashcards Preview

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Flashcards in Lec 18 Pathology of Interstitial Lung Disease Deck (47)
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1
Q

What is honeycomb lung

A

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

2
Q

What happens to compliance in interstitial lung disease?

A

decreased compliance

3
Q

What happens to lung volumes in interstitial lung disease?

A

decreased lung volumes

4
Q

What happens to pulmonary BP in interstitial lung disease?

A

get pulmonary htn

5
Q

How does fibrosis happen in interstitial lung disease?

A
  • have abnormal repair w/ replacement by dense connective tissue; loss of normal architecture; organ dysfunction
6
Q

Who gets usually interstitial pneumonia?

A

people > age 50

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

7
Q

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

A

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

8
Q

What defines idiopathic pulmonary fibrosis?

A

UIP pattern on histology PLUS idiopathic disease clinically

9
Q

What do you see on histology with UIP?

A

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

10
Q

What is clinical course/treatment of usual interstitial fibrosis?

A

treat = steroid have minimal benefit; transplant in some pts

mean survival 3 yrs or less

11
Q

What is non-specific interstitial pneumonia?

A

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

12
Q

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

A

better prognosis for cellular; has biggest response to steroids

13
Q

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

A

diffuse chronic inflammatory cell infiltrates
no fibrosis
no significant alveolar expansion

14
Q

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

A

diffuse interstitial fibrosis w/ uniform appearance

fibrosis all same age = temporally uniform

15
Q

Who gets lymphocytic interstitial pneumonia [LIP]?

A

seen in pts with sjogren’s, HIV

16
Q

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

A

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

radiologically = show diffuse ground glass changes w/ cysts

17
Q

What is clinical presentation of LIP?

A

presents as cough/dyspnea

18
Q

What is sarcoidosis? How does it classically present?

A
  • multisystemic disease of unknown origin

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

19
Q

Who usually gets sarcoidosis?

A

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

20
Q

What is prognosis of sarcoidosis?

A

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

21
Q

What are multi-system symptoms of sarcoidosis?

A

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%

22
Q

What is morphology of sarcoidosis?

A

non-necrotizing epithelioid granulomas usually tightly packed

in interstitium adjacent to bronchioles

23
Q

What is classic triad of hypersensitivity pneumonitis?

A
  • chronic bronchiolitis/interstitial pneumonia
  • poorly formed granulomas
  • organizing pneumonia
24
Q

What is respiratory bronchiolitis?

A

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

may have mild chronic inflammation and fibrosis

25
Q

What is resp bronchiolitis associated interstitial lung disease?

A

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

26
Q

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

A

more diffuse than RB-ILD and more severe

insidious onset SOB

27
Q

Who gets desquamative interstitial pneumonia?

A

disease of current or past cigarette smokers

28
Q

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

A

bilateral lower lobe involvement

ground glass infiltrates

29
Q

What is treatment for desquamative interstitial pneumonia [DIP]?

A

steroids, smoking cessation

30
Q

What do you see microscopically in DIP?

A

diffuse intraalveolar macrophages w/ fine granular brown pigment

minimal changes in alveolar septa
no significant interstitial fibrosis

31
Q

What is langerhands cell histiocytosis?

A

bronchiolocentric fibrosis w/ stellate scar formation

variable number of langerhans

32
Q

Who gets langerhans cell histiocytosis?

A

male smokers

33
Q

Birbeck granule is a sign of what?

A

tennis raque shaped intracellular structure = characterstic of langerhans cell

34
Q

What is a ferruginous body?

A

inorganic substance w/ coating of iron and protein

can be graphite, ceramic, iron, etc

35
Q

What is an asbestos body?

A

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

36
Q

What are manifestations of asbestos disease?

A
  • pleural fibrosis and/ord pleural plaques

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

37
Q

What are pleural plaques?

A

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

do not contain asbestos bodies

38
Q

What is asbestosis?

A

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

39
Q

Who is at risk for asbestosis?

A

shipyard worker, insulator, pipefitter

40
Q

Where does asbestosis begin?

A

in lower lobes subpleurally

begins around resp bronchioles/alveolar ducts ands extends distally

41
Q

What do you see in silicosis?

A
  • marked silicotic nodules that enlarge and obstruct airways and blood vessels
42
Q

Who is at risk for silicosis?

A

miners, glass manufacturers, sandblasters, stone cutters

43
Q

What do you see grossly in silicosis?

A

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

44
Q

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

A

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

45
Q

What do you see in simple coal workers pneumoconiosis?

A

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

46
Q

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

A

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

47
Q

What is caplan syndrome?

A

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