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40 Cards in this Set

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inorganic particles that cause ILD
asbestos
silica
smoking
organic particles that cause ILD
moldy hay- goes away
moldy cheese
pigeon droppign
mummy
drugs that cause ILD
amiodarone
CT disorders that can lead to ILD
RA and scleroderma
usual interstitial pneumonia UIP
seen in IPF, but also in RA scleroderma SjoS
broncholitis obliterans and organizing pneumonia
seen in COP RA PM/DM
what do you need to diagnose ILD? 3 things
clinical data
radiogrophs
pathology
History and PE
cough, SOB
drugs? chemo
joint problems, rashes, thick skin
distal clubbing
velcro rales bilateral bottom of lungs
PFT for ILD
restriction, low TLC
impaired DLCO
normal O2 at rest
what are ground glass opacities?
more inflamation mor cells, more white stuff
reversible
acute intersitial pneumonia
acut hypersensitve pneumonitis
CTD-ILD
what are reticular opacities?
lacy
some degree of scarring
IPF NSIP, collagen vascular disease, CTD ILD
what is honeycombing?
established fibrosis
dead lung, not reversible
IPF.... CTD ILD, asbestosis chrnic hypersens Pneumonia
Usual interstitial pneumonia
Worse prognosis of the histo patterns
temporal heterogenous pattern: normal old fibro new fibro
IPF
non specific interstitial pneumonia
temporally homogenous- diffuse inflmation, alveolar walls are thickened
better prognosis
common in CTDs
bronchiolitis obliterans with organizing pneumonia
excellent prognosis- treat w/steroids
patch plugs of granulation in alveoli
healling pneumonia, infection, drug rxns
granulomatous inflamation
histo patern with rim of lymphocytes aroudn multinucleated giant ells (foreign body or infection) patched or diffuse
variable prog
sarcoid, hypsens pneumonitis, infections, MTB fungus
what CTDs can cause ILDs?
RA SLE scleroderma PD/DM SjoS

mainly RA and scleroderma
what histo patterns do you see in CTD ILDs?
RA- UIP is more prevalent, but also see NSIP
SS-NSIP

NSIP most prevalent in general CTDs
better prognosis than IPF
RA with secondary ILD
worse the joints are the worse the lungs are usually RF and CCP are high with lung involve.

40% have ILD, more common in men
immunosuppressive helps some

CT- shows exensive scarr and honeycomb or ggo
Systemic sclerosis
very deadly
immune complex attacks small vessels of skin, increase collagen deposits (smooth skin),

pHTN, raynauds, esophageal dysmotility, kidney fil
no lung transplant
just like IPF
what do you see in a radiograph of someone with asbestosis
pleural plaque, pleural thickening, fibrosis of lung, round atelectasis, honeycomb
what kind of cancer is associated with asbestos
mesothelioma
how do you manage asbestosis?
prevent exposure
smoking cessation
early detection
supplemtneal O2
prompt treatment of resp infect
pneumocoocal and flu shots
STEROIDS DONT WORK
what is the most common ILD
IPF
how can you Dx IPF
HRCT
what are the top three causes of digital clubbing?
IPF, congenital HD (isenmengers) and CF
what type of histo pattern do you see in IPF?
UIP, honeycombing, no ggo
who gets IPF?
typically white men over 50
what is the median survival for someone Dx with IPF
about 3 years
do antiinflamatories help IPF
no! inflamation isnt prom in UIP.
Steroids don't help either, they hurt!

IPF may be a disorder of healing process
what is the pathophysiology of IPF
myofibroblasts never die and continue to prod. EDM that is not reabsorbed-->failiure of reepithelization of new lung tissue
what are the 5 take home points for IPF
1. most common ILD, worse prog
2. Disease of older caucasian
3. HRCT alone may Dx
4. secondary to disorderd wound healing
5. no effective Rx, consider LT ASAP
what is sarcoidosis?
chronic multi organ granulomatous inflmatory disease
10x more in AA
exclude histo and blasto
what findings suggest sarcoidosis?
young AA
noncaseating granulomas
no evidence of infection
cel ratio of CD4:CD8 > 2:1
how do you treat sarcoidosis?
immunosupressives only if there are extrathoracic involvement

ibuprofen for discomfort
what is lofgren's syndrom?
erythema nodosum in legs
hilar lymph node enlargement (lymphoma)
ankle arthritis

associated with sarcoidosis
lupus pernio
pathognomonic for sarcoidosis
indolent red lesions on cheeks nose lips not painful

poor prognosis
erythema nodosum
classic for sarrcoid, good prognosis
painful red areas btwn knee and ankle
treat with NSAIDS
what is unique about calcium metabolism in sarcoidosis
hypercalcuria= a lot of kidney stones, cure by treating sarcoid

hypercalcemia less common that uria

antingen activates alveolar macrophages--> increased production of 1a hydroxylase--> incr 1,25 Vit D (not normally made in lungs) and calcium
is high serum ACE related to sarcoidosis
yes, but not useful clinically