Lec 18 Pathology of Interstitial Lung Disease Flashcards
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
