Lec 18 Pathology of Interstitial Lung Disease Flashcards Preview

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

What is honeycomb lung

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


What happens to compliance in interstitial lung disease?

decreased compliance


What happens to lung volumes in interstitial lung disease?

decreased lung volumes


What happens to pulmonary BP in interstitial lung disease?

get pulmonary htn


How does fibrosis happen in interstitial lung disease?

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


Who gets usually interstitial pneumonia?

people > age 50

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


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

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


What defines idiopathic pulmonary fibrosis?

UIP pattern on histology PLUS idiopathic disease clinically


What do you see on histology with UIP?

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


What is clinical course/treatment of usual interstitial fibrosis?

treat = steroid have minimal benefit; transplant in some pts

mean survival 3 yrs or less


What is non-specific interstitial pneumonia?

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


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

better prognosis for cellular; has biggest response to steroids


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

diffuse chronic inflammatory cell infiltrates
no fibrosis
no significant alveolar expansion


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

diffuse interstitial fibrosis w/ uniform appearance

fibrosis all same age = temporally uniform


Who gets lymphocytic interstitial pneumonia [LIP]?

seen in pts with sjogren's, HIV


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

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

radiologically = show diffuse ground glass changes w/ cysts


What is clinical presentation of LIP?

presents as cough/dyspnea


What is sarcoidosis? How does it classically present?

- multisystemic disease of unknown origin
- classically --> interstitial/nodular lung infiltrates and bilateral hilar adenopathy


Who usually gets sarcoidosis?

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


What is prognosis of sarcoidosis?

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


What are multi-system symptoms of sarcoidosis?

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%


What is morphology of sarcoidosis?

non-necrotizing epithelioid granulomas usually tightly packed

in interstitium adjacent to bronchioles


What is classic triad of hypersensitivity pneumonitis?

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


What is respiratory bronchiolitis?

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

may have mild chronic inflammation and fibrosis


What is resp bronchiolitis associated interstitial lung disease?

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


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

more diffuse than RB-ILD and more severe

insidious onset SOB


Who gets desquamative interstitial pneumonia?

disease of current or past cigarette smokers


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

bilateral lower lobe involvement
ground glass infiltrates


What is treatment for desquamative interstitial pneumonia [DIP]?

steroids, smoking cessation


What do you see microscopically in DIP?

diffuse intraalveolar macrophages w/ fine granular brown pigment

minimal changes in alveolar septa
no significant interstitial fibrosis


What is langerhands cell histiocytosis?

bronchiolocentric fibrosis w/ stellate scar formation

variable number of langerhans


Who gets langerhans cell histiocytosis?

male smokers


Birbeck granule is a sign of what?

tennis raque shaped intracellular structure = characterstic of langerhans cell


What is a ferruginous body?

inorganic substance w/ coating of iron and protein

can be graphite, ceramic, iron, etc


What is an asbestos body?

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


What are manifestations of asbestos disease?

- pleural fibrosis and/ord pleural plaques
- pulmonary parenchymal fibrosis [asbestosis] --> end stage fibrosis + honeycomb lung


What are pleural plaques?

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

do not contain asbestos bodies


What is asbestosis?

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


Who is at risk for asbestosis?

shipyard worker, insulator, pipefitter


Where does asbestosis begin?

in lower lobes subpleurally

begins around resp bronchioles/alveolar ducts ands extends distally


What do you see in silicosis?

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


Who is at risk for silicosis?

miners, glass manufacturers, sandblasters, stone cutters


What do you see grossly in silicosis?

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


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

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


What do you see in simple coal workers pneumoconiosis?

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


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

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


What is caplan syndrome?

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