Behcet's disease
cannot be confirmed through clinical laboratory results. Usually
you will be assigned to an immunologist or rheumatologist. Diagnosis
of Behcet disease is based on clinical criteria because of the absence
of a pathognomonic laboratory test. The period between the appearance
of an initial symptom and a major or minor second manifestation
can be up to a decade long in many cases. The number of different
criteria/classification systems that have been introduced over the
past 25 years reflects the failure of any single one to meet clinical
demands.
The revised 1987 criteria of the Japanese group (Shimizu) have been
widely applied (Table 1 below). More recently,
the diagnostic criteria of the International Study Group for Behcet
Disease (ISGBD) have been applied in establishing firmer diagnoses
(Table 2 below). The major limitation of these
criteria, however, lies in the fact that recurrent oral ulceration
is the linchpin for diagnosis of Behçet disease. For example,
patients with uveitis and genital ulcer, without oral
aphthosis, would not be considered to have Behçet disease,
although this is in fact a far-advanced form of the disease. Therefore,
we recommend that the Japanese criteria be applied concurrently
with the ISGBD criteria until a more exact system is devised. Behcet's
can be called either a disease or syndrome ( combination of symptoms
indicating a particular disease).
Behcet's can also be classified under different types of 'Behcet's
Diagnosis'. Such as Vasculo-Behcet's, Neuro-Behcet's, Occulo-Behcet's
and so on so forth.
The symptoms do not
have to occur simultaneously, but you must have had mouth ulcers
with at least two other symptoms. The two other symptoms can occur
on separate occasions This is why Behcet's is difficult to diagnose.
There are other symptoms associated with Behcet's, which are gone
into detail on the 'symptoms
page'. These are arthritis, gastro-intestinal problems, superficial
thrombo-phlebitis, deep vein thrombosis, hearing and balance problems,
central nervous damage mimicking MS ( multiple sclerosis ), benign
intracranial hypertension and meningo-encephlitis. Don't panic though,
as in the majority, Behcet's tends to take a mild form. It is difficult
for your physician to make a diagnosis, and it may be the case that
you are diagnosed under the banner of 'suspected Behcet's' if your
symptoms are not clearly defined under the international diagnostic
criteria.
If you do have the classic symptoms
you will be given a definite diagnosis. This is why we stress that
if you have 'suspected Behcet's, you monitor and document 'ANY'
symptoms that you may experience, however minor these may seem to
you.
It is usually the case that to have a confirmed diagnosis of Behcet's,
you should have recurrent mouth ulcers. However about 5% of known
cases of Behcet's patients do not have mouth ulcers. It could be
possible that you experience nasal or throat ulcers, these could
come under the same category.
If you read below, you will see there are other symptoms associated
with Behcet's, but you may not necessarily experience them all.
How is Behcet's
Syndrome diagnosed?
Table
1. Diagnostic criteria of the Behcet syndrome research committee
of Japan 1987 revision Major Recurrent aphthous ulceration
of the oral mucous membrane
|
Major
|
Recurrent aphthous
ulceration of the oral mucous membrane
Skin Lesions
Erythema nodosum
Subcutaneous thrombophlebitis
Folliculitis, acnelike lesions
Cutaneous hypersensitivity
Eye lesions
Iridocyclitis
Chorioretinitis, retino-uveitis
Definite history of chorioretinitis or retino-uveitis
Genital ulcers
|
Minor
|
Arthritis without deformity and ankylosis
Gastrointestinal lesions characterised by ileocecal ulcers
Epididymitis
Vascular lesions
Central nervous system symptoms
|
Diagnosis
Complete
Incomplete
Suspected
|
4 major features
3 major features
or 2 major + 2 minor
or typical ocular symptom + 1 major or 2
minor features
2 major features
or 1 major + 2 minor
|
|
|
·
Table 2. International criteria for classification of Behcet disease
|
1990
|
Recurrent
oral ulceration
|
Minor aphthous
Major aphthous or herpetiform
ulceration observed by a physician
or reported reliably by patient
Recurrent at least 3 times
in one 12-month period
Plus 2 of the following:
|
Recurrent genital ulceration
|
Recurrent genital aphthous ulceration or scarring, especially
males, observed by a physician or reliably reported by patient
|
Eye lesions
|
Anterior uveitis
Posterior uveitis
Cells in vitreous on slit lamp
examination
or
Retinal vasculitis observed by
physician (ophthalmologist)
|
Skin lesions
|
Erythema nodosum-like lesions
observed by physician or reliably reported by patient
Pseudofolliculitis
Papulopustular lesions
or
Acneiform nodules consistent with Behcet disease observed
by a physician and in postadolescent patients not receiving
corticosteroids
|
Positive pathergy test
|
An erythematous papule, > 2 mm, at the prick site 48 h
after the application of sterile needle, 20-22 gauge, which
obliquely penetrated avascular skin to a depth of 5 mm:read
by physician at 48 h
|
What
causes the symptoms and disturbances of Behcet's Syndrome?
The exact cause of Behçet’s disease is unknown. Most of the symptoms
are caused by inflammation of the blood vessels, particularly veins.
Inflammation is the body’s characteristic reaction to injury or
disease and is marked by four signs: swelling, redness, heat, and
pain.
The
problem with Behcet's is the unpredictability of when and how major
the outbreak will be. Doctors think that an autoimmune reaction
may cause blood vessels to become inflamed, but they do not know
what triggers this reaction.
We
all have our own theory's, and one that is subscribed to is having
a dormant 'Behcet's gene. This dormant Behcet gene is awoken by
a trauma or even a series of trauma's. The trauma can range from
having a series of viral infections, which accumulated can trigger
the gene to having been subjected to high stress levels, which again,
in the right circumstance, can trigger the Behcet gene. It may also
have a connection with the bodies chemical imbalance during these
episodes.
In an autoimmune reaction, the immune system mistakenly attacks
and harms the body’s own tissues. Under normal conditions, the immune
system protects the body from diseases and infections by killing
harmful “foreign” substances, such as germs, that enter the body.
Indeed, treatments are now concentrating on introducing a 'Trojan'
germ. What this 'Trojan' germ will do, is tell the bodies defence
system to attack 'it' instead of the bodies tissues. Very
shortly we will be putting 'exclusive' details of one of
these such treatments.
( You could assimilate the inflammation to say "arthritis" in your
veins instead of in your joints).
Behçet’s
disease is not contagious and does not spread from one person to
another. Researchers think that two factors are probably important
in its development. First, it is believed that abnormalities of
the immune system make some people susceptible to the disease. Researchers
think that this problem may be inherited; that is, it may be due
to one or more specific genes. Second, something in the environment,
possibly a bacterium or virus, might trigger or activate the disease
in susceptible people. Researchers have found that people who have
frequent strep infections (caused by Streptococcus bacteria)
are more likely to develop Behçet’s disease.
We believe that each of us has a trigger. A trigger could be stress,
dietary or even externally influenced. Flair up's can follow a stressful
event and that event could be anything. Sulphur
is another.
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