Friday Facts – What is Behcet’s? (Part One)

Behcet’s is at once an “illness” that is easy to describe and yet incredibly difficult to explain. It is very much like an autoimmune disease but it is NOT a “true” autoimmune disease: it is a type of auto-inflammatory vasculitis. I have recently run into a barrage of “fire,” as it were, for questioning someone in the Behcet’s “community” when I said Behcet’s was an AUTO-INFLAMMATORY disorder, not an autoimmune disease. Of course, after my “attack” when saying this, others, who seem to MIMIC my moves but who have what seem to be “minions,” re-referenced their own definition of Behcet’s, calling it “auto-inflammatory,” rather than “autoimmune.”

What is the difference between auto-inflammatory and autoimmune? Autoimmune diseases such as Lupus, Rheumatoid Arthritis, and many others, are illnesses that occur when the body produces antibodies that attack various tissues / systems in the body – with Lupus, that can be the kidneys or any number of other organs and systems; with RA it is often most severely (but not exclusively) the joints. With all of these diseases, however, there is a large overlap in symptoms – symptoms that are necessary when diagnosing Behcet’s – things such as ulcers (anywhere there are “mucous membranes” in the body), skin ulcerations / lesions, eye involvement (uveitis, primarily, although there is a long list of eye problems any of these diseases can cause), GI involvement, CNS involvement, organ involvement, &c. It is in the symptoms of these diseases (and in the case of Behcet’s, a “syndrome”) that the similarities occur – it is in the labs where the differences occur. “True autoimmune” diseases, looked at as a whole, tend to be illnesses in which the patients’ bodies produce “auto-antibodies.” You might have heard of “ANA,” anti-nuclear antibodies – auto-antibodies that attack the nucleus of cells, thus triggering a “true autoimmune” response. The presence of ANA is estimated to occur in about 95% of Lupus patients – an incredibly high percentage. The auto-antibodies involved with RA are a little more difficult to explain, but their presence in bloodwork / labs helps diagnose a patient with what is called “seropositive” Rheumatoid Arthritis. Crohn’s is more complicated and I readily acknowledge I do not know very much about its diagnosis insofar as labs and tests go (other than findings in upper GI series and colonoscopies, &c.) – if you have Crohn’s and would like to educate me and others more on your illness, please comment below or redirect me/us to your site or another site, &c.! I for one would certainly like to know more!

At any rate, the incidence of auto-antibodies in Behcet’s is not indicative of the “syndrome.” (I realize I refer to it as a “disease” frequently here – it has become “Behcet’s Disease” colloquially, but it is, as of now, a “syndrome” – the difference between a disease and a syndrome is that a disease presents with a KNOWN set of symptoms that are diagnostically relevant; a syndrome, on the other hand, can present with one of several “clusters” of symptoms – that is, although there are overlapping symptoms that TEND to occur within patients, there are “clusters” of symptoms that tend to occur in certain patients – for example, with Behcet’s, there are (at least as far as I know!) 3 such “clusters” – the traditional, HLA-B51+ type (although not ALWAYS HLA-B51+! It gets tricky fast, I realize!) that presents as more “traditional” vasculitis wih uveitis and other eye involvement; the type that involves the CNS (central nervous system); and the type that tends to involve the GI tract. These symptoms can overlap as well – that is, a patient who suffers most severely with GI involvement may also have severe eye disease as well as CNS involvement – think of it something like a “Venn Diagram” where there are 3 major circles but each of the 3 overlaps) That is to say, that when diagnosing a patient, the presence of auto-antibodies can actually help doctors rule out Behcet’s at times, depending on other labs and other clinical manifestations. Basically Behcet’s is an INFLAMMATORY disorder that causes inflammation of the blood vessels; it is unique as a vasculitis (inflammation of the blood vessels) because it affects ALL SIZES of blood vessels, rather than others which involve only or primarily small or large blood vessels. However, there is – again, as far as I know as of RIGHT NOW – no known specific auto-antibody that would help “diagnose” Behcet’s in the way ANA helps diagnose Lupus.

Phew! I realize that was a rapid-fire assault of information, and I hope I was clear and I hope all of my recalled information is “correct.” I have much more to discuss and I am trying to get to that ASAP here in this blog, including (re-discussing) the diagnostic criteria for Behcet’s and discussing the aforementioned “clusters,” as well as discussing the “treatments” used for this “syndrome.” I hope you are all having a happy and healthy day wherever you are in the world and I hope to be back here SOON SOON SOON!

❤ Always, Beth