List of eponymous medical treatments

Transcription

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