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FEMA
this is Eric Strong from Stanford University
and today are we talking about vasculitits. After watching this video
you should be able to define vasculitis. Describe typical features suggesting
vasculitis
describe the modern classification system of vasculitis
and most importantly identify a probable vasculitis.
in a patient with atypical presentation 0
this video will not review the specific criteria the
individual forms a vasculitis that's because this will take an hour to do
would be incredibly boring and its unnecessary since the criteria is
easy to look up when needed. Instead
I'll be focusing on vasculitis as the general category a disease
particularly how to recognize a patient who probably has vasculitis
since the varied multisystem presentation can be easy to confuse
with other diseases
So first what is vasculitis?
Vasculitis is that they burst category of inflammatory diseases of the
blood vessels
these diseases range in severity from self-limited
dermatological additions to acute and rapidly paid almost a system diseases
All forms of vasculitis
are characterized by endothelial damage into more proliferation
thrombosis and eventual vascular occlusion
last letters can affect every organ system
typically in back in as both patterns based on the size of the affected
vessels
and the underlying pathologic mechanisms all
so what do those recognizable patterns look like
the first two layer a pattern recognition has to do with the size of
the involved bustles
and the knowledge that basket latest tends to affect by
organ systems the most which other skin gastrointestinal system
can these no system and the muscles
it can also affect the lungs which I've left of here because it's not common
other when it does happen its involvement is usually very prominent
when a patient has a basket like this
affecting the small blood vessels here she can get a skin condition
called palpable purpura which show a picture up in a minute
GI involvement leads to me go siders
and minor GI bleeding the GLA merry Lai
the kidneys are affected which most commonly manifest as he maturity a
without red blood cell casts and proteinuria
patients can get a pollyanna Robert P and the muscle involvement
result in my algiers which is a fancy word for muscle pain
a medium vessel vasculitis
a result into skin conditions called earthy Manado some
and livid over to kill aris patients get abdominal pain
and rarely pal preparations in the kidneys
damage is not limited to the commercial I'm so now there may be
mature your with RBC casts along with flank pain
from ischemia in the brain
patients can get a wide variety of mental status changes
from somnolence psychosis along with strokes
and in the muscles they get my own status
which differs from I'll just and that there is a usually lab
and pathologic evidence of muscle damage such as an elevated creatine kindness
a large vessel vasculitis can result in cyanosis
and discoloration of the extremities balan park Shin
hypertension from involvement of the aorta
or renal arteries but no key Macharia
strokes from involvement other crowded and claudication
which is pain in the extremities due to lack of blood flow unfortunately
the presentation a basket by this is much more complicated than this
because no one individual vasculitis disease
leads to all the findings in anyone column all
there are however certain clinical features that are highly suggest to the
basket like this
the first to something called Mon underwriters multi-plex
which is a simultaneous or sequential
dysfunction a individual non-contiguous peripheral nerves
in a seemingly random pattern this typically presents as loss of sensory
and/or motor function with an individual nerves over days to weeks
for example this patient pictured involvement up the
owner might result in weakness of the fourth and fifth fingers
involvement of the lateral cutaneous from raw nerve might result in past
teachers over the lateral thigh
and involvement other common peroneal nerve could result in full drop
all in addition to basket by this other causes upon and rightist multi-plex
include diabetes *** and alloy dosis
another feature that is highly suggested the vasculitis occurs when an
unknown multisystem disease as very prominent involvement up the long and
kidneys
especially the combination of pulmonary hemorrhage and I the renal failure
and/or he Macharia as the only other Donbass tonight this diagnosis
typically does this is anti GBM antibody disease
also known as good pastors disease all
livid over to kill aris which is a place like reddish depart role
discoloration of the skin is frequently seen in medium
and occasionally small vessel vasculitis
it certainly not Pathan demonic however as an idiopathic form seen young women
is the most common etiology this finding
finally is palpable purpura which is the development
up numerous raised non blanching purplish
lesions on the skin which accused the most prominent in the lower legs
ankles and Pete as shown here
in addition to these poor clinical features there are a variety of other
features which are less specific
but are also commonly seen a basket like this
they include headache hypertension
pulmonary hemorrhage without being on Parliament
Obama pain abnormal urinary sediment
a skin condition called earthy Manado some which consists
up multiple red tender not short on the shins
arthralgia is and claudication all
here's a picture apparently min ago some all
although I said that I wouldn't be reviewing individual diagnostic criteria
of vasculitis
it's important to be familiar with the general classification scheme
unfortunately the classification of vasculitis is confusing and
unsatisfying due to many reasons including overlapping presentations
it makes a primary and secondary forms a vasculitis
evolving understanding of the underlying pathophysiology is
an evolving consensus regarding use of eponymous names
and varied opinion on whether classification should be based primarily
on the size of the affected vessels
or underlying pathologic process
as a consequence different sources may present slightly different
classification schemes
the following classification is largely based on the most recent recommendations
from the American College of Rheumatology
the first order division within vasculitis is based on that size would
target muscles
large muscle vascular disease include talk to ya issues
arteritis which predominantly affects the great vessels
of the aortic arch and as a predilection for young asian women
and giant cell arteritis also known as temporal arteritis
which primarily affects branches at the crowded arteries and which is seen
almost exclusively among the elderly
medium vessel vasculitis includes polyarteritis todos a
which is the most widely distributed within the body the vascular disease
as it can affect any organ callous sake disease
affects almost solely young children and is best known for causing coronary
artery aneurysms
and primary CNS vasculitis into mystery mimicking
all kinds of primary nor logic and psychiatric disease
small vessel vasculitis is further subdivided into that which is associated
with an
auto antibody called anti new to fill cytoplasmic antibody
and that which is associated with immune complexes
thank associated vasculitis includes three diseases
with long and frustratingly similar names
granulomatosis with poly and gids which is a particularly
dangerous vasculitis primarily affecting the Kines
longs upper airway nose and eyes
houston affiliate granulomatosis with poly and gids
which is characterized by the combination asthma sinus problems
drop at the and peripheral your sin filial
and microscopic poly and gids which is clinically very similar
to granulomatosis poly and gids the exception to the locker upper airway
involvement
and the lack of granulomas on biopsy all
the final category opinion complex associated vasculitis
includes I G a basket by this it typically self-limited disease see
mostly in children
which is characterized by pop opera abdominal pain
astrologers and renal involvement usually limited to
Macharia while globulin the mic basket lettuce which is caused by
antibodies called while globulins which precipitate when the temperature drops
below body temperature
most cases are triggered by chronic hepatitis C infection
and last hypersensitivity vasculitis
which is usually limited to the skin but can also cause Bieber
arthralgia stand accused limp and not the
it can be triggered by medications particularly penicillins and
cephalosporins
along with a variety of chronic infections
a quick word about some %uh those names some types a basket like this
previously had different opinion based names for example
granulomatosis with poly any ideas was known as Wagoner's granulomatosis
until just a few years ago houston affiliate granulomatosis with poly and
gids
was known as church Strauss syndrome and I G a basket by this was known as he
knocked shoreline
purpura impact most positions they were paired to these diseases by the
eponymous
even if the scientific literature shying away from them
journals and professional societies aren't just changing the names for the
sake of avoiding
opinions and general instead Wegener's granulomatosis was named after
feat Rick Wagoner was a Nazi doctor was speculated to participate in experiments
on concentration camp prisoners
but it seems that once one opinion was intentionally removed from the formal
ex-con
all the others are following it seems likely that copy of his
arteritis and Kawasaki disease will both be renamed
in the near future
in addition to the types of primary vasculitis just listed
some systemic diseases can trigger a secondary vasculitis
for example connective tissue diseases malignancy
chronic infection sarcoidosis
and bishops disease the last which is often considered
a primary basket latest itself
there are also many diseases which can mimic basket like this
including PIC Berger's disease
house people axis cholesterol Emeli Sande rum
anti GBM antibody disease bacterial endocarditis
and amyloidosis some sources
actually list Berger's disease and anti GBM and the body disease
as true masculinities highlighting the overlapping
any incompletely understood pathophysiology evolved these disorders
so what are the general steps to diagnosing a basket latest
first the Commission must identify a collection of clinical findings which I
this is just about the latest in general
or optimally wanted to specific masculinities
second one should check with you labs in clinic why collation tasks
and a urinalysis and consider checking imaging studies
and/or anti-nuclear feels like a plastic antibodies
this will help to now down the differential diagnosis to one or two
specific vascular disease
to search for an Associated systemic on this that would make the basket by the
secondary
and to rule out vasculitis mimics all
finally the diagnosis to the basket latest should be confirmed
preferrably with biopsy of the clinically affected organ and/or
and geography all
in the final minute out very quickly review the basic treatment options for
vasculitis
although you should keep in mind that each individual about the latest may
have specific treatment algorithms
at this extremely brief overview may not capture all
for limited 18 years past the latest for example
mild I G a basket latest or hypersensitivity vasculitis
sometimes observation and removal offending agents
are all that is necessary the antihistamines
and a short course a penicillin is often used all
for mild systemic vasculitis prime his own
on the order of 0.5 to 1 milligram per kilogram per day is common
finally a rapidly progressive and life-threatening basket lettuce
Prime is own plus they say the toxic aged such as cyclophosphamide
is the most common regimen cyclophosphamide can be transition to a
less toxic agent such as
is a biplane or methotrexate after remission
is achieved
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