Behcet disease, is a rare disorder and most
of the symptoms are thought to be a result of an autoimmune process involving the blood
vessels – it’s a type of vasculitis. Among the family of disorders that cause vasculitis,
Behcet’s is fairly unique because it causes inflammation in blood vessels of all sizes
– small, medium, and larges ones – on both the arterial and venous side of the circulation. The underlying cause of Behcet’s is unknown,
but there are a number of clues. The biggest clue is that the human leukocyte
antigen, or HLA, genes seem to play a role, and this is based on the fact that having
a specific type–the HLA-B51 type–predisposes individuals to having Behcet disease. HLA genes encode proteins found on the surface
of immune cells, and play a key role in regulation of those cells, and since the disease is a
result of an autoimmune process, it makes sense that the HLA-B51 proteins could be involved. Another, clue is that an individual’s response
to viral and bacterial infections may be involved. For example, some individuals with Behcet’s
generate relatively high levels of antibodies to Helicobacter pylori which may go on to
damage blood vessel walls. This is an example of molecular mimicry where
an antibody to a foreign pathogen starts to cross-react and damage the person’s own
tissue. Individuals with Behcet’s also seem to have
a weakened innate immune system, a higher proportion of autoreactive T cells and activated
neutrophils which destroy healthy tissue, and altered levels of T helper cells and cytokines. In sum, the clues span genetic and environmental
factors and both the innate and adaptive immune system. When looking at the blood vessels in particular
– the classic finding is seeing lymphocytes in the walls of capillaries, veins, and arteries
of all sizes making them inflamed and boggy; sometimes the inflammation can get so severe
that the tissue around the vessel begins to die off completely. These changes make the endothelial lining
more likely to develop blood clots or aneurysms. Since the inflammation is happening in blood
vessels of all sizes throughout the body, individuals with Behcet’s can develop a
wide-range of symptoms. Having said that, most individuals initially
present with recurrent oral ulcers. Relative to aphthous ulcers, the oral ulcers
of Behcet disease can be larger, more painful, and take a few weeks to heal – sometimes recurring
before a previous round of ulcers has resolved, which means that they can be almost continuously
present. The genital ulcers that develop are similarly
painful and can develop around the anus, vulva, or scrotum. Eye inflammation can also develop, and can
present a number of different ways. One is anterior uveitis which causes a painful
decrease in vision, redness of the conjunctiva, and hypopyon which is also known as sterile
pus because inflammatory cells enter the anterior chamber of the eye even though there is no
infection present. Another is posterior uveitis which causes
a painless decrease in vision and visual field floaters. On rare occasions, there can also be other
types of eye inflammation including involvement of the optic nerve itself. Not surprising – any sort of eye inflammation
can potentially lead to vision loss and is therefore one of the most serious aspects
of the Behcet’s. Some individuals can also develop skin conditions
like folliculitis or erythema nodosum – an inflammation of the fat cells underneath the
skin. As Behcet disease develops, it can vary quite
a lot between individuals since it can affect every organ system. Some individuals may have joint stiffness
and abdominal pain while others develop meningitis and kidney disease The clinical diagnosis is often made based
on an individual having a pattern of symptoms consistent with the disease, in particular
recurrent oral and genital ulcers, as well as eye and skin lesions. There is no specific blood work that’s typically
done, but one interesting and unique test that can be used is the pathergy test. In a pathergy test, an individual is pricked
on the skin and then reassessed 1-2 days later. Normally, a small pin prick would heal, but
many patients with Behcet disease don’t heal normally, and instead develop a skin
lesion or ulcer at that spot. Treatment for Behcet disease is typically
geared at suppressing the immune system to help alleviate the symptoms. That includes the use of medications like
corticosteroids, biologic agents that block the effect of tumor necrosis factor, and even
intravenous immunoglobulin which can alter the immune system’s activity. Okay – let’s recap, Behcet’s is a rare
autoimmune disease that affects small, medium, and large arteries and veins, as well as capillaries. The underlying mechanism is unclear, but blood
vessels show a pattern of lymphocyte infiltration and surrounding tissue necrosis. Classical features include recurrent oral
and genital ulcers, uveitis, and skin lesions, but the disease can affect nearly every organ
system. Thanks for watching, you can help support
us by donating on patreon, or subscribing to our channel, or telling your friends about
us on social media.
thank you.
I love you
I was just asked today if I have ancestry from Turkey…is that also a similar factor to those diagnosed with Behcet's disease?
Thank you.
รญ dont know Who you are….. but whoever you are god bless you, keep it up. thanks
Please make also a video about DVT/VTE.
God bless you
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thank you. ..keep it up ๐
this disease found by Turkish scientist, Dr.behcet. just to inform:))
Can you make a video for Chiari Malformation?
Can you make a video about antibiotics
The reading is the illnes is not BIลET it id BEH รET
Behรงet is not autoimmundisease . It is inflammatr disease
I wish there was a PDF of each disease with the slides presented here all one page for each disease. Would anyone else like this? I'm an osmosis subscriber by the way. Thank you and keep it up!
just proud being turks:)thanks from turkey, alot:)
awesome guys! please more autoinmune stuffs
best tutorial channel for medicine
it's HLA B5 not B51
thanks for posting this has affected my eyes and this is some awful stuff…..its been a hard journey for me. thanks for sharing. #bechetsawareness
pathergy test is done by intra dermal injection of saline
God bless you, from Korean med student
How is the diagnosis made and how are the diagnosis diferrentials discarded?
Is there a cure?
G
i just looooooooooove your channel
bay – sets
10 days…that is when I go for a brain biopsy to confirm if I have this. Fingers crossed i don"t…then it's only ADEM to deal with
The quality of illustrations/visuals and depth of information you guys have in each of your videos never ceases to amaze me. Osmosis is an invaluable resource, thank you!!
Behcet syndrome is an autoimmune disorder and if you guys type in Rosie marcel she is a British actor mainly known as jac naylor from Holby city she has it and there is a clip about her explaining it
Thank you very much
Thank you๐๐
Thank you
thank you for finally explaining to me my disease. no doctor has been able to tell me exactly what was happening but just told me i had this. thank you so much
Vaccines cause this ๐
Great channel! Find it very helpful & have used it more than once in my studies as a med student…Only one small correction: it's ''Adamantiades-Behcet'' Disease…
I thank you so very much.
Your easy explanations are amazing.
For all others if you want current updated info on this terrible illness, search behcet with ncbi.
That's where the most updated research usually can be found.
Can any one explaine how this is deferred from systemic lobus?
Thanks for explaining in a more technical way instead of just summing up the common things. I was just diagnosed after years and years of trouble this disease has caused me. Luckily for now- not much changes in eyesight. It was noticed by a doctor finally, when I was recently admitted in hospital for sepsis and I just happened to mention to the dr my ulcers on my privates (which seem to flair when I get sick) after testing negative for every STD they could think of, taking into consideration my long history of GI problems and bleeding, as well as constantly battling different ailments from swollen painful ankles, mouth ulcers, strep like symptoms but never testing positive for strep, tonsillitis, or mono, ect (the long list) we've finally discovered whats been making me suffer for so long. Its hurt relationships, people have accused me of lying, complaining too much, its cost me jobs… just… bleh.
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I don't know who you are… I don't know what your name is.. but I'm going to find you.. and going to hug you ! ๐
Great video! thanks for it
I want to thank you for making this video I have bichette syndrome and it's so hard trying to explain or show somebody how bad the syndrome really is and what it can do to to a person.
If I could take back all the money I paid for med school and donated it to you, I would
I have this, im 14 and i was diagnosed when i was 10. Its a living hell… plus i also have scoliosis and kyphosis and disautonomy. I fucking hate this
Behcet patient here! For treatment medicines that contains colchium is being widely used, as well.
Fellow sufferer here . May God Bless you if your thinking you may have this . And if not also . Keep fighting . I have it 17 years now . God help the people suffering now who are only learning about this as they arenโt helping with pain meds now . Thank God I had this before or they wouldnโt help me I would be dead from my own hand . Those pain meds keep me alive . I fear for people, who suffer and donโt have cancer . They think only cancer cause s sever pain .
I have bahjet disease ๐ญ๐ญ๐ญ
Thank you for making this video. I have Behcet's, and I am going to share this so my family and friends can understand a little bit more. Again, thank you.
thank you for young the correct term VULVA… usually this should be expected from people who work in the medical field, but most people use the wrong term…
You are great
Behรงet's disease is named after a Turkish scientist, Prof. Dr. Hulusi Behรงet.