Vasculitis – An Overview

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 HIV 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 for FEMA there in

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