Restrictive Lung Disease - Path, Gupta Flashcards
definition of restrictive lung dz - signs, symptoms
heterogenous group of dz characterized by dyspnea and reduced TLC
what is the buzzword for the appearance of end stage lung dz in restrictive dz
honeycomb
what disease is a result of repeated cycles of injury caused by a yet unidentified agent resulting in diffuse fibrosis?
Idiopathic Pulmonary Fibrosis
presentation of idiopathic pulmonary fibrosis
insidious, DOE, dry cough
gradualy deteriorating course
treatment for IPF?
none current
how do you diagnose IPF?
by exclusion - you must rule out secondary causes of fibrosis such as drugs and radiation
what is the prognosis of IPF?
bad
in IPF, what is the appearance of the fibrosis? where is it found?
patchy interstitial fibrosis
predominantly lower lobe, subpleural distribution
will have areas of normal lung tissue intervening
**It is heterogenous in time and space - different areas of damage are at a different stage in the dz process than other areas
what are some key microscopic findings in IPF
fibroblast foci, collagenized areas, mild lymphocytic inflammation
what is the pathogenesis of IPF? - what is the key player in fibrosis
TGF-beta - from injured penumocytes induces fibrosis
in IPF, where is the concentration of fibrosis the highest - what is its distribution?
subpleural - towards periphery
what happens to the epithelium of bronchi in IPF
metaplastic change - goes from more PSSCE to squamous epithelium
for IPF, when you look at it histologically, what is it called?
usual interstitial pneumonia (UIP)
if you see an intraalveolar pattern of fibrosing, rather than interstitial, what dz are you thinking?
cryptogenic organizing pneumonia
in cryptogenic organizing pneumonia, compare the age of the various areas of organizing connective tissue
all of the same age (diff from IPF)
what is the treatment for cryptogenic organizing pneumonia
steroids
what must you differentiate cryptogenic organizing pneumonia from? (i.e. what dz process has a similar histologic pattern)
resolving acute lung injury/ infection - like pneumonia, or ARDS
what is a defining histologic finding in cryptogenic organzing pneumonia?
intraalveolar plugs of loose fibroconnective tissue
what is the name of a non-neoplastic lung reaction to inhaled dust, chronic exposure
pneumoconioses
what are 4 examples of dusts that can cause pneumoconioses
coal
silica
asbestos
beryllium
define anthracosis
what dz process do you see them in
Innocuous inhaled carbonaceous pigment engulfed by macrophages that accuulates in connective tissue along lymphatics and in lymphoid tissue
seen in Coal Workers Pneumoconiosis
in simple CWP, what is the pulmonary function? where do you find it in the lung?
little/no pulmonary dysfunction
upper lobe predominant
what is the pathogenesis of complicated CWP, and what is the lung function?
there is a progression from simple to complicated CWP over many years
leads to progressive massive fibrosis
will compromise lung function
what is histologic finding of complicated CWP
black scars composed of pigment and dense collagen
what is a high yield association for complicated CWP - another dz
rheumatoid arthritis
what is epidemiology associations for silicosis (pneumoconiosis caused by inhalation of silica)
sandblasters, mine workers, stone cutters
what is the histologic pathology of silicosis, and where in lung do you find it?
nodular fibrosis, mostly in upper lobes and hilar nodes
what is the specific pathophys of silicosis - i.e. what causes the fibrosis
silica ingestion by macrophages causes release of fibrogenic mediators - results in hard collagenous nodules
what is a diagnostic technique you can use to visualize silica in silicosis
polarizing microscopy demonstrates birefringent silica particles
what does silicosis increase your susceptibility to and why
TB
likely related to impaired function of macrophages
for pneumoconiosis due to Beryllium (Berylliosis?) - what is the histopathologic finding and where in what tissues do you see it
noncaseating granulomas
seen in lung, hilar lymph nodes and systemic organs
what does berylliosis leave you at increased risk for
lung cancer
taking a good pt history is necessary to differentiate what disease from berylliosis?
sarcoidosis
also has noncaseating granulomas in lung and systemic organs
(look at question stem for epidemiology shit)
besides being a beryllium miner (damn unions), what other high yield epidemiology is associated with berylliosis
space industry
Like Jeezy said, me I’m in a spaceship, that’s right I work for NASA
name the 4 non-neoplastic asbestos-related diseases
1) diffuse pleuarl fibrosis
2) benign pleural effusions
3) pleural plaques
4) parenchymal interstitial fibrosis (Asbestosis)
what are the two types (shapes) of asbestos fibers, and which one is more harmful
1) Serpentine - curly flexible fibers
2) Amphibole - straight stiff fibers - more pathogenic, less easily ingested by macrophages
what are pleural plaques (result of asbestos) and where are they found anatomically
circumscribed plaques of dense collagen
most often arise on parietal pleura and diphragmatic domes
Parenchymal Interstitial Fibrosis (Asbestosis) - what is the time frame and pathogenesis - including what parts of the lung/airway
slowly progressive, heavy prolonged asbestos exposure, latency period 20+ years
initial injury to respiratory bronchioles and alveolar ducts, macrophages attempt to ingest and clear debris, release fibrogenic mediators - fibrosis of adjacent alveoli
How do you differentiate Asbestosis from usual interstitial pneumonia (fibrosis) - upon looking at a histo slide
presence of asbestos bodies
involve the body coating asbestos fibers with iron stuff
golden brown beaded rods w/ translucent fiber cores (this will be question)
what is a method you can use to better determine if asbestos is present in a histo slide
prussian blue stain
stains the iron containing proteinaceous material surrounding the asbestos fibers (ferrigenous bodies)
(know this also)
good pt history is impt to know to try this stain
what 2 drugs do you need to rule out as causes of fibrosis in the lung
1) Bleomycin
2) Amiodarone
what other therapeutic process can causes fibrosis in the lungs
radiation,
especially to the chest
what are features of a histo slide containing drug induced penumonitis
mild interstitial fibrosis
lymphocyte inflammation
penumocyte hyperplasia
what is the defining characteristic of sarcoidosis
non-necrotizing granulomatous inflammation
systemic disease of unknown cause
what areas are affected by sarcoidosis (3)
hilar lymph nodes
lungs
skin
what are some signs/symptoms of saroidosis
insidious onset of SOB
cough
and/or constitutional symptoms
what is treatment for sarcoidosis
steroids
what is typical epidemiology of sarcoidosis
black female
sarcoidosis - what does BAL fluid show - for inflammatory cell types
increased CD4:CD8 ratio, greater than 2.5
due to inc CD4 (helper) T lymphocytes
just know that sarcoidosis is disease of exclusion
good good
buzzword for histopathology of sarcoidosis
non-caseating epitheliod granulomas
(Must know)
presence of giant cells
sarcoidosis - what is the buzzword for the common giant cell inclusions (2)
how specific are they for sarcoidosis?
asteroid bodies
Schaumann bodies - laminated calcified concretions
not very specific
what is the name for an immunologically mediated lung disorder due to prolonged exposure to inhaled organic dusts
hypersensitivity pneumonitis
what type of hypersensitivity is hypersensitivity pneumonitis (chronic form)?
type IV (delayed type)
for hypersensitivity pneumonitis, what leads to fibrosis? how can you prevent fibrosis?
chronic exposure to inhaled antigen leads to fibrosis
removal of environmental antigen prevents progression to fibrosis
what is the buzzword for epidemiology for hypersensitivity pneumonitis
pigeon breeder’s disease
i.e. exposure to inhaled antigens from birds
what is another (not birds) buzzword epidemiology for hypersensitivity pneumonitis
farmer’s lung
from moldy hay
what are 3 histopathologic features of hypersensitivity pneumonitis
airway-centered interstitial lymphocytic inflammation loosely formed ill-define granulomas interstitial fibrosis (not eosionphils)
what is the end stage microscopic appearance of hypersensitivity pneumonitis
honeycomb
Desquamative Interstitial Pneumonia (DIP) - classified as what type of interstitial disease
smoking-related interstitial disease
What are the 2 histopathologic features we need to know regarding desquamative interstitial pneumonia
1) temporal and spacial uniformity - everything everywhere looks the same on slide
2) alveolar spaces are completely full with macrophages that have a golden brown color
what disease is associated with a BRAF mutation
Langerhans Cell Histiocytosis
what is the epidemiology of Pulmonary Langerhans Cell Histiocytosis
95% young smokers
what is histopathologic finding with Pulmonary Langerhans Cell histiocytosis
coffee bean shaped cells
and eosinophils
what is the pathogenesis and histopathology of Pulmonary Alveolar Proteinosis
defect in macrophages that causes accumulation of surfactant in the alveolar spaces
alveoli are filled with dense, amorphous, protein-lipid granular precipitate
alveolar walls are normal
what other pathology appears like Pulmonary Alveolar Proteinosis, but looks more fluffy and is caused by a fungus
Pneumocystis pneumonia
causative organism pneumocystis jirovecii
histo - looks more fluffy, bubbly, whatever (the shit accumulated in the alveoli)
what stain do you use for pneumocystis and what is the appearance
Gomori silver stain
look like crushed ping pong balls
what pts are susceptible to pneumocystis
immunocompromised hosts
Muthiah - what is the most common cause of idiopathic interstitial pneumonia
usual interstitial pneumonia AKA idiopathic pulmonary fibrosis
Muthiah - what is the most common interstitial lung disease with no know cause
IPF / UIP
Muthiah - for UIP / IPF - what is the histo apperance with regards to age and distribution
there is injury happening at different times
Muthiah - what is the location of IPF / UIP w/in the lung (lobe, and space)
lower lobe
subpleural
Muthiah - what is the common pt w/ IPF
caucasian male, smoker, 6th or 7th decade
Muthiah - UIP can be seen in many other diseases besides IPF - what makes UIP in IPF different
IPF have a progressively worsening course
Muthiah - what is another pathology that can be associated with IPF and what environmental exposure
GERD
Wood dust exposure
Muthiah - what antibody is associated with Rheumatoid arthritis
Rheumatoid factor Anti-CCP
Muthiah - what antibody is associated with systemic sclerosis
Scl 70
Muthiah - what antibody is associated with mixed connective tissue disease
anti-ribonucleoprotein (RNP)
Muthiah - antibody associated with dermatomyositis / polymyositis
anti - Jo-1
Muthiah - what is the most common cause of interstitial lung disease overall? which ones do we need to remember? (2)
hypersensitivity pneumonitis
we probably miss half the diagnoses
bird fancier’s disease
farmer’s lung
Muthiah - how to tell apart berylliosis and sarcoidosis
Berylliosis - exposure to beryllium
aerospace industry, ceramic tile workers, beryllium miners
Muthiah - does asbestos exposure cause more mesothelioma or bronchogenic carcinoma?
bronchogenic carcinoma
Muthiah - what 3 drugs can cause pulmonary fibrosis
bleomycin, amiodarone, methotrexate
Muthiah - what are key factors for silicosis
1) type of lung disease
2) lobe predominance
3) exposure to what/how
1) interstitial
2) upper lobe predominant
3) silica - sandblasters, stone cutters
