Could it be Behcet's Disease?

Marlène

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http://www.behcets.org.uk/menus/main.asp?PN=medical-information-for-patients-how-diagnosed

Diagnostic guidelines

There are levels of certainty for diagnosis:
  1. International Study Group diagnostic guidelines (very strict for research purposes)
  2. Practical clinical diagnosis (generally agreed pattern but not so strict)
  3. ‘Suspected’ or ‘Possible’ diagnosis (incomplete pattern of symptoms)
1. International Study Group strict research level guidelines for diagnosis

Must have:
  • mouth ulcers (any shape,size or number at least 3 times in any 12 months)
Along with 2 out of the next 4 ‘hallmark’ symptoms:
  • genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)
  • skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)
  • eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
  • pathergy reaction (papule >2 mm diameter, 24-48 hrs or more after needle-prick)
2. Practical clinical guidelines for patients not included in research cohorts

Must have:
  • mouth ulcers
Along with 1 out of the 4 ‘hallmark’ symptoms above
Along with 2 of the following symptoms:
  • arthritis/arthralgia
  • nervous system symptoms
  • stomach and/or bowel inflammation
  • deep vein thrombosis
  • superficial thrombophlebitis
  • cardiovascular problems
  • inflammatory problems in chest and lungs
  • problems with hearing and/or balance
  • extreme exhaustion
  • changes of personality, psychoses
  • any other member of the family with a diagnosis of Behçet’s disease
3. 'Suspected' or 'possible' diagnosis

Usually given when someone does not have mouth ulcers or has mouth ulcers but does not have 1 of the 4 'hallmark' symptoms but has other symptoms and signs of inflammation and other causes for these have been ruled out.
 

Marlène

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Tissue-type HLA-B51

There has been some interest in the tissue-type marker B51, as a statistical link exists between people with Behcet's disease being positive for that type and having inflammation in the eyes. However, this only applies to people from Japan, China and the Middle East and is not apparent in people of Western ethnic origins. This limits its usefulness as a diagnostic marker. Research interest is now directed away from the B51 locus to adjacent areas on the same chromosome, with the possibility that there may be a malfunction in a gene or genes which form part of the immune system there.

http://www.behcets.org.uk/menus/main.asp?PN=medical-information-for-patients-similar-disorders
 

Marlène

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Some similar disorders (differential diagnoses)

  • Ulcerative colitis
  • Crohn's disease
  • Coeliac disease
  • HLA-B27 seronegative arthritis - eg ankylosing spondylitis
  • Reiter's syndrome
  • Stevens-Johnson syndrome
  • Sarcoidosis
  • Multiple sclerosis
  • Immune deficiency diseases
  • Systemic lupus erythematosus (SLE, lupus)
  • Rheumatoid arthritis
  • Sjögren's syndrome
  • Lyme disease (Borrelia burgdorferi)
  • Food intolerance and allergy
http://www.behcets.org.uk/menus/main.asp?PN=medical-information-for-patients-similar-disorders
 

merylg

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Tissue-type HLA-B51

There has been some interest in the tissue-type marker B51, as a statistical link exists between people with Behcet's disease being positive for that type and having inflammation in the eyes. However, this only applies to people from Japan, China and the Middle East and is not apparent in people of Western ethnic origins. This limits its usefulness as a diagnostic marker. Research interest is now directed away from the B51 locus to adjacent areas on the same chromosome, with the possibility that there may be a malfunction in a gene or genes which form part of the immune system there.

http://www.behcets.org.uk/menus/main.asp?PN=medical-information-for-patients-similar-disorders
Hi Marlene, yes I read this statement you highlighted but have found evidence that this is not the case. There have been studies done in Ireland on HLA B51: http://bjo.bmj.com/content/81/8/649.full

Just out of interest my gene snp results from 23andMe tested:

rs3024490 ... (applicable ethnicity European)...AA Moderately higher odds of Behcet's Disease
rs1800871... (" " Asian) .............AA Typical odds ie not lower odds
rs1495965 .... (" " Asian)..............CC Moderately higher odds of Behcet's
 

merylg

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Good list of differential diagnoses thanks Marlene. I am HLA B-27 Neg and RF Neg yet have some features like Seronegative Spondyloarthropathy/Psoriatic arthritis. I am at increased gebetic risk for Coeliac Disease, but blood tests & biopsy Negative. Increased genetic risk for Ulcerative Colitis. No evidence of Colitis from numerous routine colonoscopies. No apparent Sarcoidosis yet ACE is slightly raised. MS excluded by MRI. Lupus? but ANA Neg. Sjogren's excluded at least on blood tests.
Food Intolerance & Allergy: YES
Lyme disease??? Possible (Neg blood tests cannot exclude, diagnosis is clinical)
Behcet's Disease??? Possible
Lupus??? Possible
Primary Biliary Cirrhosis??? Possible http://au.wiley.com/WileyCDA/PressRelease/pressReleaseId-102342.html :thumbsup:
Other??? Possible
 

Marlène

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Several microbial antigens have been shown to stimulate T effector lymphocytes in Behçet’s Disease patients, e.g., staphylococcal antigens, streptococcal antigens Escherchia coli-derived peptides and Chlamydia pneumoniae. Heat shock proteins (HSP) of various microbes could be involved in the pathogenesis of Behçet’s Disease. A cross-reactive response to human HSP60 was suggested as causative in Behçet’s Disease.
...
This has led to the possibility that Behçet’s Disease is not an autoimmune disease but rather should be classed as autoinflammatory, a distinction that should change our investigations into the causative mechanisms of Behçet’s Disease.
http://www.behcets.org.uk/Documents/web - scientific.pdf

Well, I happen to have the ulcers since childhood but never paid attention to it, just part of my life,
I have horrible daily headaches since the age of 6,
doctors never agree whether I have lupus or not,
I have huge antibodies against chlamydia pneumonia (Cpn) and negotiating with my doctor to get AB treatment
Not to mention the list of classical symptoms we all share

Maybe I have to consider Behcet for once and get a clue what went wrong.
Secondly, if it is not an autoimmune disease, then antibiotics could maybe eradicate the Cpn as explained at cpnhelp.org

So glad this forum exists ...
 

alex3619

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Interestingly as as a teenager I would have fit the clinical but not research definition of this. I had unending mouth ulcers - usually three or four at the same time. Something was clearly up with my immune system. However as an adult I do not fit this diagnosis. So the question arises: are there a spectrum of such disorders? Is ME one of the diseases on that spectrum? Bye, Alex
 

Sushi

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Interesting about the mouth ulcers! All my life I had been plagued with them -- like Alex, lots of them, and almost all the time.

They disappeared entirely with GcMAF treatment which might mean that it just improves the immune system (not sure if GcMAF would affect autoimmune?) or maybe that it is killing some pathogen involved in the mouth ulcers. I am curious about this too.

I don't have any of the other markers for Behçet’s Disease.

More ideas?

Sushi
 

merylg

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I had mouth ulcers as a teenager, some as an adult, none now. Somehow with Behcet's there is a connection to ulcers in gut too but it's not part of the classic criteria for the diagnosis. I have had stomach ulcers & non-specific atrophic gastritis, which is like patchy areas (H. pylori Neg on biopsies).

I never had more than one mouth ulcer at a time, and they were small. For some years now have had nasal septal ulcers that heal temporarily with topical antibiotics then come back again. Rheumy seemed to think this all relevant, but we have a few other possible diagnoses...having some more tests...sigh...

On a trial of Arava (which I halted) I got a boil in my armpit & my liver was affected by the drug. I seem susceptible to skin infections. In 2010 I had minor scratch on scalp from a hair-clip & I developed an infection, then cellulitis and lymphadenitis which spread from lymph node on top of head to lymph node at base of skull.
Other times I have had boils & folliculitis. For a long time I had infected hives on back before I found out they were a reaction to coffee :( Perhaps Group A Strep playing a part here too as well as Staph. & maybe Propionebacterium?

Also once had Group B Strep as a coloniser. GP not worried. Have since had antibiotics for other reasons so hoping it is gone anyway. Rather not have it as part of my normal flora!

Something interesting from 23andMe is I have a genetic variant that means I make 1/3 the amount as most people, of IL-10 important for good immune response.
 

merylg

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I paid to have HLA - B51 typing test done (no govt subsidy of this test) . If it is POS it will add some weight to a possible diagnosis. If Neg the diagnosis is still possible, as not everyone with Behcet's is POS.

(From what I have been told it is not an easy disease to treat & the treatments seem like trial and error of all the kids of things we are trying here!)

Sushi - Interesting about your experience on GcMAF, with the ulcers disappearing. Did you have any increase in inflammation? Or was that controlled by using small doses? Monitoring Nagalase levels?

alex3619 - Interesting thoughts about possible spectrum of disorders...but one disease. Something else that Dr Borody says is that "there are no disease states that are caused by a single infective agent"


Marlène - Thanks for all the information :thumbsup:
 

Sushi

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...
Sushi - Interesting about your experience on GcMAF, with the ulcers disappearing. Did you have any increase in inflammation? Or was that controlled by using small doses? Monitoring Nagalase levels?
... Thanks for all the information :thumbsup:
Hi merylg,

I did have an increase in inflammation on GcMAF--mainly IL 8. I am able to control it by taking low doses. Nagalase has steadily gone down for me.

Best,
Sushi
 

justy

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Mouth ulcers here too as a child and teenager - once so severe my whole mouth and lips were covered overnight. I also had constant severe conjuctivitis as a child. Still get occasional mout ulcers now, and like Meryl the nose ulcers too. Not sure if i have any of the other symptoms though.

I hope you get some answers from your rheumy soon - he seems very keen to consider a proper diagnosis - whilst here in the UK cant get anyone interested in anything.

All the best, Justy x
 

justy

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Just read through Marlenes list above and i have also had on a number of occasions, spots in the lady region that wouldnt heal (not herpes)

I also had my legs waxed once and developed terrible foliculitis when the hiars started to grow back - i was left with very sore and spotty legs whilst on holiday. I also regularly get sores on my scalp, which i thought could be foliculitis.

Sounds like another disease to put on my list to cross off.
Justy x
 

Marlène

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If Behcet's attacks the veins (large and small ones) then:
- I should consider the rupture of a vessel and blood transfusion during an intervention about 10 years ago.
- when I was very sick but still working I collapsed two times choking out of the blue. Now I read symptoms are is similar to lung aneurysm.
- recently went back to the gyne because of relentless pain in lower belly and blood loss, he said my belly was full of varicose veins.
- sometimes I have vasculitis on my lower legs, darkblue spot necrosing in the middle
- I had eye infections for years when I was a teenager
- I tried ginseng panax once and it triggered a huge attack (influences blood circulation)
...

It still does not explain my sun sensitivity, MCS, PENE, adrenal burnout, low thyroid values, low VIP, low CD57, ...
unless someone else has an idea?
 

Marlène

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Different phases (I, II, III) and treatment (traditional and with supplements) of Behcet's explained

http://www.angelfire.com/ma/Behcetbook/shapiro.html
Behcet's Disease: Immune Process and the Potential Impact of
Nutritional Supplementation and Pharmaco-Nutrition


Example:
In one study (Niwa et al 1985), patients showing an increase in neutrophil active oxygen generation, including those with Behcet's, rheumatoid arthritis, Crohn's disease, and progressive systematic sclerosis, were treated with injections of liposomal superoxide dismutase (SOD), 2.5 mg twice a week. There was a marked reduction in 12 out of 16 patients with active Behcet's disease, with particular effectiveness against intestinal manifestations. Remission rates in other diseases were 7 out of 8 mucocutaneous lymphnode syndrome / Kawasaki disease, 3 of 5 dermatitis herpetiformis, IgA linear bullous dermatosis or severe cement dermatosis, 4 out of 9 severe and active rheumatoid arthritis, 3 out of 3 PSS, 4 out of 4 Crohn's disease, 3 out of 4 colitis ulcerosa, and 2 out of 2 unresponsive (hemolytic) anemia. Several terminal-stage PSS patients showed dramatic improvement. The researchers reported no toxicity from liposomal SOD, which had various advantages compared to free SOD preparations.
 

merylg

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If Behcet's attacks the veins (large and small ones) then:
- I should consider the rupture of a vessel and blood transfusion during an intervention about 10 years ago.
- when I was very sick but still working I collapsed two times choking out of the blue. Now I read symptoms are is similar to lung aneurysm.
- recently went back to the gyne because of relentless pain in lower belly and blood loss, he said my belly was full of varicose veins.
- sometimes I have vasculitis on my lower legs, darkblue spot necrosing in the middle
- I had eye infections for years when I was a teenager
- I tried ginseng panax once and it triggered a huge attack (influences blood circulation)
...

It still does not explain my sun sensitivity, MCS, PENE, adrenal burnout, low thyroid values, low VIP, low CD57, ...
unless someone else has an idea?
yes I get sun sensitivity, eyes sensitive to bright light & sunshine, severe MCS, PENE, feel like I have adrenal burnout but don't, but I do have liver issues....seem to have Primary Biliary Cirrhosis happening at same time, which IS documented but rare. I also seem to have Psoriatic arthritis too & ?EDS type 6 and ?MCAD too... remember there is a pathogen involved somehow as a cause/trigger ?perpetuator of the autoimmune vasculitis of medium arteries and veins. Attacks genetically susceptible people. Other things like Myeloperoxidase Deficiency would compound the susceptibility to infection. Also any primary or secondary immune deficiencies that one might have.

http://www.ncbi.nlm.nih.gov/pubmed/16610072

http://www.ncbi.nlm.nih.gov/pubmed/6743363