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Behcet's Disease in King
Hussein Medical Center
.........................................................................................................................
Manal Mashaleh MD1, Yousef
M. Ajlouni, MD2, Abdallah Serhan MD3, Imad Ghazzawi
MD4
From Division of Rheumatology, Department of Internal
Medicine, King Hussein Hospital. Royal Medical
Services, Amman-Jordan
1 Senior Rheumatologist
2,4 Consultant Gastroenterologist
3 Senior Internist and intensive care
Correspondence:
Manal Mashaleh
Rheumatologist
Department of Internal Medicine
King Hussein Hospital
Tel: 0779382007
Manal_mashaleh@yahoo.com
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ABSTRACT
Objectives:
To study how frequently we diagnose cases
of Behcet's disease at King Hussein Medical
Center, and what are the most common manifestations
and the diagnostic tests used.
Material and methods: This Study
was conducted as a retrospective survey
on epidemiologic and clinical features
of patients with Behcet"s disease
diagnosed at King Hussein medical center
in Amman from 2004 to 2007. 107 patients
were diagnosed with Behcet's disease when
they fulfilled the International study
group criteria for the diagnosis of Behçet's
disease.
Results: The sex ratio was 1:3.3
with male predominance (82 male and 25
female). Clinically, 97 % had oral ulcers,
87 % had genital ulcers, 92 % had skin
lesions and 32 % had ocular lesions. 36%
had vascular, and 21% neurological manifestations.
Arthritis was seen in 60%. The arthritis
was intermittent, self-limiting, and non-destructive,
involving large joints, mainly the knees
and ankles. The pathergy test showed positive
in 33 % of patients. HLA-B51 wass positive
in 48 and negative in 26 patients while
undetermined in 33 patients.
Conclusion: Behcet's disease is
not uncommon in Jordan so we must be more
aware of its existence. Male predominance
is prominent in our patients. Genital
ulcer and ocular lesions were the most
frequent manifestations. Positivity of
pathergy test was not a frequent finding
as in other studies done in different
world countries.
Key words:
Behcet's syndrome, Genital ulcer, Ocular
lesions, pathergy test, HLA-B51.
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Behçet's disease
(BD) is a multi-systemic disorder of recurrent
acute inflammation. It is named after Hulusi
Behçet, a Turkish dermatologist who,
in 1937, recognized recurrent oral and genital
ulcers and iridocyclitis as a separate clinical
entity,1 although symptoms similar to those
of BD were described as early as the time of
Hippocrates and later by several others.2-4
The prevalence of the disease varies widely
from country to country and probably from race
to race. The disease is more common in the Middle
and Far East than in Europe and the USA.5
The major characteristic of Behçet's
disease (BD) is a systemic vasculitis, with
the mucosa, skin and eyes being predominantly
involved7. Although BD may affect almost any
system of the body with exacerbations and remissions
of inflammation, the key clinical manifestations
are recurrent oral aphthous ulcers, genital
ulcers, uveitis and skin lesions, such as erythema
nodosum, pseudofolliculitis, papulopustular
lesions and acneiform nodules8.
HLA-B51 has been the most closely associated
risk factor for BD9. There is no specific
laboratory tests and pathognomonic findings
in BD; therefore, the diagnosis remains largely
a clinical one. It should be kept in mind, however,
that recurrent oral ulcers are reported in almost
all patients with BD10-12.
In the last 2 decades we have become more aware
of the existence of BD in Jordan. In this study
we aimed to determine how frequently we diagnose
cases of Behcet's disease at King Hussein Medical
Center, what are the most common manifestations
and the diagnostic tests used.
King Hussein Medical Center
is a teaching hospital, and receives referrals
from all medical sectors in different parts
in Jordan. It serves the armed forces personnel
and the independents.
A total of 107 patients who visited the hospitals
in the period between 2004 to 2007 were included
as having Behcet's disease according to the
International study group criteria for the diagnosis
of Behçet's disease (Table 1)13.
The following data were collected from medical
records and the patients themselves: patient's
name, date of birth, sex, geographic distribution,
clinical manifestations, durations of the symptoms,
and positivity to pathergy test. Duration of
symptoms was determined as the period from the
beginning of at least one of the diagnostic
symptom criteria to the patient's first visit.
Geographic distribution was based on the location
of patients' residency. Full clinical examinations
were performed. Arthritis was defined as joint
tenderness, swelling, and/or pain on motion.
The following tests were carried out on all
patients by standard methods: complete blood
picture; erythrocyte sedimentation rate; urine
analysis; antinuclear factor test; rheumatoid
factor test; serum uric acid; C reactive protein;
serum proteins and electrophoresis; X-rays of
the chest, hands, feet, pelvis, and any other
affected joint. The ESR were regarded as raised
if above 20 mm/lst h. C reactive protein readings
were recorded as either positive or negative.
Pathergy test was performed with oblique insertion
of a 20 gauge needle under sterile conditions
and read by a physician at 48 hr. Formation
of papule or pustule was interpreted as a positive
result. Tissue typing was performed on patients
with BD by the standard NIH microcytotoxicity
technique using antisera for A, B, and C locus
antigen.
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Table 1 Adapted
from International Study Group for Behcet's
disease. Criteria for diagnosis of Behcet's
disease13 |
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Major criteria (need 1) |
Recurrent oral ulceration |
Minor aphthous, major aphthous, or herpetiformis
ulceration observed by physician or patient
that recurred at least three times over
a 12-month period |
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Minor criteria (need 2) |
Recurrent genital ulceration |
Aphthous ulceration/scarring observed by
physician or patient |
|
Eye lesions |
Anterior or posterior uveitis or cells in
vitreous on slit lamp examination; or retinal
vasculitis observed by ophthalmologist |
|
Skin lesions |
Erythema nodosum observed by physician or
patient, pseudofolliculitis or papulopustular
lesions; or Acneiform nodules observed by
the physician in a post adolescent patient
who is not receiving corticosteroid treatment |
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Positive pathergy test |
As interpreted by physician at 24 to 48
hours |
107 patients (82 male and 25 female) were included
in the study. Geographical distribution showed
the highest frequency in north of Jordan (69%)
as in Table 2. The sex ratio was 1:3.3 with
male predominance. Their ages ranged from 18
to 48 years with a median age of 18 (Table 3).
Clinical manifestations are summarized in Table
4 and were: had oral ulcers (97%), genital ulcers
(87%), skin lesions (92%), Arthritis (60%),
Gastrointestinal manifestation (36%), ocular
lesions (32%), vascular (36%), and neurological
manifestations (21%).
Definite arthritis was observed in 60% of patients,
but arthralgia was reported more commonly. Arthritis
was oligo- or polyarticular, intermittent, self-limiting,
and non-destructive, involving peripheral joints,
the knee being involved most frequently. Gastrointestinal
complaints were reported by 36%, these being
vomiting, abdominal pain, flatulence, diarrhoea,
or constipation.10 patients had pulmonary involvement
in the form of pulmonary aneurysm in 3 patients
and 2 with pulmonary emboli. No renal involvement
was detected.
The pathergy test was done for all patients
and showed positive in 89 patients (83%) and
revealed negative in 18 (17%). The HLAB51 test
was done for 69 patients and showed positive
in 49 patients (71%) and revealed negative in
20 (29%). There was no positive association
between eye involvement, arthritis, HLA-B51
tissue typing, and positive pathergy test.
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Table
2 Geographical distribution of patients |
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Area |
No of patients |
Percentage |
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North of Jordan |
74 |
69% |
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Middle of Jordan |
24 |
22% |
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South of Jordan |
9 |
0.08% |
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Table
3 Number and ages of patients at presentation |
|
Age range |
No of patients |
Percentage of patients |
|
10-19 |
12 |
11% |
|
20-29 |
52 |
48% |
|
30-39 |
37 |
35% |
|
40-49 |
5 |
5% |
|
50-59 |
0 |
0% |
|
60-69 |
1 |
1% |
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Table
4 Clinical features in 107 patients
with BD |
|
Clinical feature |
Number of patients |
Percentage |
|
Oral ulceration |
104 |
97% |
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Genital ulceration |
93 |
88% |
|
Skin manifestation |
99 |
92% |
|
Eye involvement |
34 |
32% |
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Arthritis |
64 |
60% |
|
Neurological manifestation |
22 |
21% |
|
Gastrointestinal manifestation |
39 |
36% |
|
Vascular |
39 |
36% |
Behçet's disease
(BD) is a multi-systemic disorder of recurrent
acute inflammation. The disease may affect almost
all/any systems or organs, including the ocular,
pulmonary, gastrointestinal, genitourinary and
nervous systems, as well as joints and large
vessels. Nevertheless, the major characteristic
of the disease is a systemic vasculitis with
mucosa, skin and the eyes being predominantly
involved.
The highest prevalence of the disease has been
reported in Turks living in Anatolia (Northeastern
Turkey) with 370 patients per 100,000 inhabitants14, while the overall prevalence in Asia is
20- to 30-fold lower and in Europe and the U.S.A.
more than 150-fold lower15-18.
Interestingly, in areas with many ethnic populations,
certain ethnic groups were found
mainly affected as for example in Kuwait. Kuwaiti
Bedouins were not affected by the disease and
there was only a prevalence of 1.58 per 100,000
Kuwaitis which was similar to the involvement
of non-Arab populations (1.35 per 100,000 inhabitants),
while 2.90 per 100,000 non-Kuwaiti Arabs were
affected19.
In populations with the same ethnic origin as
in Jordan the prevalence of the disease seems
to be strongly dependent on the longitude or
the latitude of their residence. The prevalence
in Jordan populations was calculated to increase
up to 3-fold by going to the North (Table 2).
Our study shows that sex ratio was 1:3.3 with
male predominance which is similar to reports
from other Arab countries such as Saudi Arabia,
Iraq, Kuwait, and Jordan, and disclosed a male
to female ratio of BD of 1.2:1 to 3.4:1.The
clinical expression of BD as well as HLA-B51
association are also similar to that found in
most of the series studied in those countries
in the percentage of patients with genital ulcers,
skin manifestations, and central nervous system
involvement.20-23
As in other studies, the positive rates of major
symptoms revealed that the most frequent symptoms
were oral ulcers and articular. Moreover, the
positive rate of ocular lesions in the present
study was much higher than that in the previous
studies in Jordan. The increase in frequency
of ocular lesions may be due to the increase
in routine tests for the eyes since the ocular
lesions are considered to be one of the most
important prognostic factors.
The positivity of the pathergy test in the present
study (33%) was significantly higher than that
of Japan (43.8%) and China (62.2%).24,25
This observation may be attributed to the differences
in the method of pathergy test or to the racial
differences. The positivity of pathergy test
is especially high in the Middle Eastern countries,
which made the test as a crucial parameter of
diagnosis of Behcet's disease.
In conclusion, Behcet's disease remains an elusive
disorder, having variable clinical presentations
and unknown etiology, and lacking any specific
therapy. However, in view of the establishment
of well-defined diagnostic clinical criteria,
it should now be possible to investigate the
cause and therapy of this potentially serious
disorder with greater focus than was previously
possible. Behcet's disease is not uncommon in
Jordan so we must be more aware of its existence.
The male predominance is prominent, higher frequency
of genital ulcer, ocular lesions, and positivity
of pathergy test in the patients. The positivity
of the pathergy test and the possession of the
HLA-B51 antigen differ from country to country
and that positivity of the pathergy test is
not related to the presence of the HLA-B51 antigen.
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