Follow-up discussion regarding 2013 research article about diagnosing Behcet's using a point system

I'm always pleased to see further refinement of the diagnosis classification systems for Behcet's. It's particularly important to have robust definitions in order to identify who should be included in wider research studies (such as the ongoing Behcet's studies at NIH and around the world).

However, this does not mean that patients who don't meet the 4 points threshold don't have BD and therefore should be treated as such. As we all know, Behcet's doesn't just pop up with all its symptoms presenting at once. Often it can take years of monitoring unexplained symptoms for a doctor (or patient) to suspect BD as one of the differential diagnoses.

Plus, the ICBD point system does NOT take into account other Behcet's symptoms that can add weight to the picture such as various gastrointestinal problems, nonspecific rashes, cardiac and pulmonary inflammation, family history, recurrent headaches and memory/confusion problems, HLA-B51 test, unexplained fevers, extreme fatigue, and positive response to medications.

Treatment can be based on a physician's suspicion of "possible Behcet's," "probable Behcet's," and even identifying Behcet's as a "working diagnosis for now." However, these patients would not ordinarily be included in research studies because that may skew the results. That does not mean that over time, these patients won't continue to develop more symptoms and finally meet the criteria for "complete Behcet's."

Sometimes effective and aggressive treatment can mask or prevent additional symptoms from appearing. If you're on a medication that's working well, you may appear to be in some sort of remission or only display a couple mild recurring BD symptoms. This can confuse the picture for a doctor trying to make a firm diagnosis.

Table 6 in the full 2013 article defines a patient scoring 1 or less as "almost certainly NOT having Behcet's" (at this time), while scoring a 2 equates to Behcet's being "very unlikely" (at this time). A score of three points means "possible, but unlikely Behcet's" (at this time). The researchers placed the minimum score for probably having BD at a score of 4. A 5 meant "highly likely," and a 6 equates to "almost certainly" having Behcet's.

None of these scores included a positive pathergy reaction, so if that test was taken and repeatedly positive, another point could be added to the score. Perhaps (my suggestion) an extra half point could be included for each common, recurrent "other BD symptom" as mentioned above.

With the strict scoring system, I currently (cumulatively) score 7 points, but if I add in bonus half points for my GI, pulmonary, extreme fatigue and positive response to BD medications, I'd give myself a 9.

However, there have been short periods of time when my symptoms were well controlled with Trental, high dose prednisone, or Enbrel and a rheumatologist actually questioned my diagnosis. Sometimes you just have to ignore a doctor's shortsighted or disbelieving comments and continue on the course you know is right. That's where documentation, photos, and record keeping help so much.

Eventually, many of the BD drugs will "wear out" even with higher doses or may cause intolerable side effects, and the disease's symptoms will come back. Some symptoms may be more aggressive in the first 5-10-15-20 years of the disease; others can pop up for awhile and then disappear, never to reappear.

I still score my two diagnosed mild bouts of retinal vasculitis even though they have not reappeared for the past 20 years. Similarly, I had two eruptions of erythema nodosum early on in my case and those have not recurred for 15 years. It's not uncommon for genital ulcerations to pop up much less often than oral ulcers, but that doesn't exclude them from the diagnosis.

I am not bothered by the newly proposed point system. It offers a framework and admits some of its shortcomings. What the 2013 article doesn't make clear, however, is that the diagnosis of Behcet's isn't a one-shot YES or NO or even MAYBE deal. It's a process over a number of years which can include other less-common symptoms of the disease and requires elimination of alternate possibilities.

I once had a compassionate neurologist who could not confirm that my atypical migraines (which caused confusion and tremors) was due to Behcet's neurological involvement because all MRI's came back as normal, but she told me she was going to treat them "AS IF" the headaches were due to CNS involvement because of my diagnosis and the sense that flares of other BD symptoms correlated with my migraine flares.

This was a breath of fresh air after I had been seeing a neurologist who pointed to the negative MRI and claimed, "Pictures never lie." Now we know that MRI's often fail to spot subtle Behcet's neuro inflammation and that perhaps a SPECT scan would have demonstrated the reasons behind my CNS symptoms.

Finally, keep in mind that classification and diagnostic criteria are not the same thing, but there can be overlap or thought of as a continuum. For a more in-depth discussion of this, refer to Dr. Hasan Yazici's 2009 article: nyuhjdbulletin.org/Mod/Bull...

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  • Hi

    How well you have made your comment. I have suffered BD for over 26 years but only diagnosed after 12 years. My main Rheumy consultant still has doubts and has put these on record which in some ways has cost me dearly. Still I am luckier than most in that I have not suffered as much degeneration, on the other hand although not severe I generally have some manifestation each month. Still I have muddled through be it because of my own inner strength ( having to bring up 3 children almost alone), and at times being aware enough to stand my ground. I read the article also...interesting.

    Hugs Billi

    ps. Got my first neuro appt next week......I shall think of your doc!!!!

  • Thank you for your thoughtful, informative post. I, too agree with any expansion helping assist medical professionals in accurately diagnosing and treating Behcet's, & hope that can be done without obscuring the diagnostic picture for people who - like me - have had full evidence of their disease necessary for a complete diagnosis manifest over a longer time period. My initial mention of Behcet's was in 2003 as a differential diagnosis to UCTD. At that time, I had positive pathergy, rashes, oral lesions, genital ulcerations (tested & confirmed not to be herpetic or other STD's), a positive "autologous serum" test response (for which I was put on and responded well to methotrexate, prednisone, & very high dose allergy medications @20x standard dose). I also had cardiac problems, an abnormal brain MRI, migraines, chronic idiopathic uriticaria (rashes) & angioedema with dermatographia. Lab work was abormal sometimes (high CRP & ESR), completely normal for a year at a time other times. HLA tests have always been negative. I reached a 1st full renission in late 2004, with Behcet's still my differential diagnosis. My 2nd pregnancy triggered a terrible flare in 2006. By 2007, I was so sick, for a little while they thought I had Wegener's. Luckily, they ruled that out & pretty quickly landed solidly on definitive Behcet's. That diagnosis has not been questioned since (except by some very rude dermatologists who failed to discover vasculitis in the particular area they biopsied). At that point, I had lesions the size of apples on my face half the time, & up to 70 at any given time all over my body (even in my ears at times). I was grateful for the doctors who told me to ignore the rude ones & go somewhere where they knew what they were doing with Behcet's. I did not have my 1st bout of Iritis until 2011 when I had 3 episodes involving my right eye. I have also lost partial hearing on one side & facial sensation & symmetry on the right. (Neuros are still reluctant to confirm NBD. What they do say is "a long history of neuro problems probably related at least in part to her Behcet's." Right now, I am struggling with increasing problems with involuntary movement, tremors, etc., but working on ruling out medication contributors 1st.) My newest issue is adrenal insufficiency, possibly related to polyendocrine autoimmune..., but I haven't found anyone in my town who seems to be able to tell me if that is from the Behcet's or what it all means or what to expect now -- So anyway, long way of saying, yes, symptoms can take a long time to develop & it is hard to find good doctors who know how to read them (even the good ones who want to be able to be helpful) - so whatever can help guide the medical professionals who help us, I'm all for!

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