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Prevalence, types, and characteristics of headache in involvement of the central nervous system in the Syrian population” a case-control study

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  • Damascus hopital

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Introduction Oral ulcers, genital ulcers, and uveitis represent the typical trial of Behcet’s disease (BD). It is well common on the Old Silk Road. The mucocutaneous lesions are the hallmark of BD, but neurological involvement is one of the severe symptoms. Headaches may be an early sign of BD neurological involvement. This study aims to investigate the headache prevalence and its types, and characteristics in a Syrian sample of BD patients. Methods BD patients were clinically interviewed and examined to collect their information, symptoms, and signs. the International Study Group for Behcet’s Disease diagnosis criteria was used to confirm the BD diagnosis. The International Classification of headaches was used when classifying the headaches. Results 120 participants were included in the study. half of them were BD patients and the control group was also 60 participants. Among Syrian BD patients, 36.7% suffer from primary headaches and 36.7% suffer from secondary headaches. These findings were not significantly different between the BD patients and the healthy population. Our results showed that there was no statistically significant difference between the two groups. Conclusion Headache should not be considered a predictor for neurological involvement among BD patients. Additional attention to BD patients or the specific treatment for headaches is not required and does not differ from the general population.
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Prevalence, types, and characteristics of headache
in Behcets disease without involvement of the
central nervous system in the Syrian population:
a case-cohort study
Karam Rabah, MDa, Nour Rabah, MDa, Haya Deeb, MDb, Ghina Haidar, MDc,*, Maysoun Kudsi, MD, PhDc
Introduction: Oral ulcers, genital ulcers, and uveitis represent the typical trial of Behcets disease (BD). It is well common on the Old
Silk Road. The mucocutaneous lesions are the hallmark of BD, but neurological involvement is one of the severe symptoms.
Headaches may be an early sign of BD neurological involvement. This study aims to investigate the headache prevalence and its
types, and characteristics in a Syrian sample of BD patients.
Methods: BD patients were clinically interviewed and examined to collect their information, symptoms, and signs. the International
Study Group for Behcets Disease diagnosis criteria was used to conrm the BD diagnosis. The International Classication of
headaches was used when classifying the headaches.
Results: One hundred twenty participants were included in the study. half of them were BD patients and the control group was also
60 participants. Among Syrian BD patients, 36.7% suffer from primary headaches and 36.7% suffer from secondary headaches.
These ndings were not signicantly different between the BD patients and the healthy population. Our results showed that there was
no statistically signicant difference between the two groups.
Conclusion: Headache should not be considered a predictor for neurological involvement among BD patients. Additional
attention to BD patients or the specic treatment for headaches is not required and does not differ from the general
population.
Keywords: Behcets disease, primary headache, secondary headache
Introduction
Bechetsdisease(BD)wasrst described by Hulusi Behcet in
1937, who identied the trial of symptoms: oral ulcers, genital
ulcers, and uveitis
[1]
. BD tends to affect younger ages between
20 and 40 years of age and less frequently in children
[2]
,
with a signicant prevalence among the people from the
Mediterranean, Middle East and Far East. It has been
named silk road diseaseforthisreason
[3]
. Both genders can
be affected by BD; male dominance is found in Arab
populations, whereas female dominance is evident in China,
Korea, and Northern European Countries
[4]
. No data about
the prevalence and characteristics of BD are available in Syria.
The mucocutaneous lesions are the hallmark of BD, but the
most severe symptoms are uveitis, large vessels, and neurological
involvement
[5]
.510% of BD patients have central nervous
involvement, most of which is parenchymal involvement. The
prevalence of headaches in BD varies, as some researchers con-
sidered it a prominent complaint of many patients, rather than a
neurological symptom
[68]
. Some patients complain about occa-
sional, mild headache, while others reportedly experience daily
disabling severe headache.
HIGHLIGHTS
The aim of this research is to investigate the headache
prevalence and its types, and characteristics in a Syrian
sample of Bechets disease (BD) patients compared to non-
BD patients.
Headaches may be an early sign of BD neurological
involvement. Among 60 Syrian BSD patients, 36.7% suffer
from primary headaches and 36.7% suffer from secondary
headaches. These ndings were not signicantly different
between the BD patients and the healthy population.
Additional attention to BD patients or the specic treat-
ment for headaches is not required and does not differ from
the general population.
a
Faculty of Medicine, Syrian Private University,
b
Faculty of Medicine and
c
Department
of Rheumatology, Faculty of Medicine, Damascus University, Damascus, Syria
Sponsorships or competing interests that may be relevant to content are disclosed at
the end of this article.
Published online 6 March 2024
*Corresponding author. Address: Rheumatology, Faculty of Medicine, Damascus University,
Universitys location: Almazzeh Street, Damascus 96311, Syria. Tel.: +963 991 898 337.
E-mail: gtghinahaidar@gmail.com (G. Haidar).
Received 2 September 2023; Accepted 24 February 2024
Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. This is an
open access article distributed under the terms of the Creative Commons
Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is
permissible to download and share the work provided it is properly cited. The work
cannot be changed in any way or used commercially without permission from the
journal.
Annals of Medicine & Surgery (2024) 86:25492554
http://dx.doi.org/10.1097/MS9.0000000000001903
Cross-sectional Study
2549
There are theories concerning headaches higher prevalence in
BD patients. Its classied as a primary headache such as tension-
type headaches, and migraines, and a secondary type of headache
such as uveitis and retinitis. In addition to specic types of
headache in patients with BD, which is not classied as primary
and secondary and are called non-structural headaches
[3]
.
Many researches have previously investigated the character-
istics and specic treatments of headache in BD; however, the
pertinent studies have thus yielded conicting results
[6,7,9,10]
. The
percentage of headache was 35%and 65%, respectively
[6,7]
.
Considering this disparity and the location of Syria on the Silk
Road, we aim of this research is to investigate the headache
prevalence and its types, and characteristics in a Syrian sample of
BD patients.
Materials and methods
Study design and sample size
A case-control study was conducted in the Rheumatology
Department of Damascus Hospital, from February 2019 to June
2020 on 120 participants, 60 BD patients, and 60 healthy
control.
The sample size, when calculated using the website (openepni.
com), was at least 55, with a condence interval of 95%, in
each group.
This study was approved by the ethics committee of Damascus
University. All patients were fully informed and agreed to the
concept and aim of this study.
We conrm that all methods employed in this study were
carried out by the relevant guidelines and regulations.
Study population
The total number of participants was 120, 60 BD patients and 60
healthy people.
The control patients were matched in age, sex, and socio-
economic characteristics. This control group participants were
referred to the hospital and were selected from patients in urology
and orthopaedic clinics, without previous medical history or
receiving any treatment. We asked many participants if they ever
had headaches, and if yes, they were enroled in our study.
Inclusion criteria
BD patients older than 18 years old who were diagnosed
depending on the BD classication criteria of the International
Study Group
[7]
.
Exclusion criteria
Pregnant women and women with gave birth within the last
6 months, patients with other connective tissue diseases, or other
auto-immune diseases, central nervous trauma and/or diseases, a
history of papilledema, psychological stress and/or disorders,
liver disorders, renal diseases, malignancy and any other dis-
orders that can cause headaches, were also excluded.
Methods
Age, sex, previous medical and surgical records, and medications
were asked and collected for all participants. The headache
characteristics and duration between headache occurrences were
collected and registered to all participants. BD duration was also
collected only for BD patients. The headache characteristics
according to the International Classication of Headache
Disorders
[3]
and the time between headache occurrence and
diagnosis of BD.
The patients were interviewed by a rheumatologist , and each
patient was individually interviewed and examined.
Headaches related to BD were classied as structural if they
started 6 months before the onset of BD or after the disease onset
which did not meet the criteria of the International Headaches
Society (IHS) for primary headaches. Any other types of head-
aches were sent to a specialist for additional investigation, except
for patients with reliable medical records. Patients with a con-
rmed diagnosis of neuro-bechet disease by magnetic resonance
imaging and cerebral spinal uid analysis were excluded. The
most severe and characterized headache was considered if a
patient had complained of more than one type of headache. All
patients and control had undergone physical examination
including a full neurological examination by the same examiner.
All patients and control had fundoscopy because, in patients with
papilledema, vision is usually not affected initially.
Study tools
International Study Group for BD diagnosis criteria
[11]
. These
criteria dened BD as recurrent oral ulceration plus two of the
following manifestations: genital ulcers, ocular lesions, derma-
tological manifestations, and a positive pathergy test.
The International Classication of Headaches
[12]
which dened
primary headaches include tension-type headaches, migraines
with/without aura, and cluster headaches. Secondary headaches
include ocular-related headaches conrmed by an ophthalmolo-
gist, infectious or sinusitis headaches, and structural headache
which is neither primary nor secondary.
Our study is compliant with The STROCSS 2021 checklist
Guideline
[13]
, and this work is submitted on the research registry
dashboard.
Data analysis
IBM Statistical Package for the Social Sciences (SPSS) software for
Windows version 28.0 was used to statically analyze the data. In
this analysis, α=0.05 and β=0.1. Also, 11% difference in
headache prevalence between the two groups was considered.
Pvalue less than 0.05 was considered signicant. We used stu-
dentsttest for comparison of quantitative data and χ
2
test for
comparison of qualitative data.
Results
Demographic data
One hundred twenty participants were included in the study. Half
of them were BD patients (18 females and 42 males) and the
control group was also 60 participants (20 females and 40 males).
The age of the participants was between 28 and 41. The total
mean of the participants age was 33.84 ( ±3.31). The mean age of
BD patients was 33.48 ( ±3.28), compared to a control group of
34.20 ( ±3.33). The mean systolic blood pressure was sig-
nicantly different between the case and control groups (125.1 vs.
121.7 mmHg; P=0.007), but within normal limits, as none of
our patients or controls previously had hypertension. There were
Rabah et al. Annals of Medicine & Surgery (2024) Annals of Medicine & Surgery
2550
not any signicant differences in sex and age groups between the
case and control group (Table 1).
The symptoms of BD patients
The most common symptoms were the three main BD symptoms:
100% oral aphthous, 60% genital ulcer, and 55% ocular mani-
festations. However, the prevalence of other symptoms was 50%
arthralgia and arthritis, 40% erythema nodosum and vascular
manifestations. Pseudo-folliculitis and gastrointestinal manifes-
tations were less frequently reported by our BD patients (30%
and 11%, respectively) (Table 2).
The prevalence and characteristics of the headache between
the two groups
The total prevalence of different types of headaches was 36.7%
and 21.7% in the case and control groups, respectively.
There was not a signicant difference between primary head-
aches between patients with BD and healthy people. The pre-
valence of primary headaches is higher in patients with BD
(36.7%), compared to healthy people (21.7%), and the most
common cause of primary headache was tension headache
among the participants (Table 3).
Moreover, Secondary headaches had a same prevalence of
36.7% in case group and anterior uveitis was the most common
cause of the secondary headaches with 28.3% (Table 4).
The relation between disease duration, and the onset of the
headache
In fty percent (50%) of patients, the headache onset was found
to be years after the onset of the disease. On the contrary, 20% of
the patients recalled having headaches years before the diagnosis
of Bechet disease was conrmed.
Consumption of medications, and family history
BD treatment
The prevalence of headaches in BD patients was not signicantly
different between prednisolone-received patients at a dose of
7.5 mg/kg daily and those who did not consume prednisolone
(P=0.10), although the prevalence of headache was about 2
times higher in patients receiving 7.5 mg/kg daily and more.
Headache medication
Additionally, 12 out of 60 patients with BD needed treatment for
their primary headache symptoms, whereas only 6 in the healthy
group needed treatment. However, there was no statistically
signicant difference between the case and control group. Also,
while comparing the family history between patients with BD and
healthy people the Pvalue was =0.42, therefore, there was no
signicant difference between the two groups. However, it was
signicantly higher in participants complaining of headaches in
both groups (Table 5).
Discussion
BD is a multiorgan disease, it affects the younger population
especially patients in their second to fourth decades, but it can
present at any age
[3,4,6]
. This increased prevalence in the Old Silk
Road and family aggregation strongly suggest the genetic factor
in inducing the disease, and the carriers of HLA-B51/B5 are at
higher risk of developing BD compared to non-carriers
[14]
. This
conrms with our results; the total mean is 33.84 ( ±3.31), and
our country Syria is located on a Silk Road.
The dominant gender in our study is male which also con-
rms the fact that men are dominant among BD patients in the
Arab population
[4]
.MostoftheclinicalsymptomsofBDare
believed to be due to auto-inammatory vasculitis, it is well
known that BD can involve arteries and veins of all sizes and
shapes
[5]
. Those vasculitis lesions of BD are without necro-
tizing vasculitis or giant cell formation
[11]
. Additionally, BD
patients lack specic auto-immune antibodies like the ones
seen in other auto-immune disorders like rheumatoid arthritis
or systematic lupus erythematous
[5]
.
The frequent early manifestations of neurological involvement
in BD are headache, fever, disorientation, poor balance or
stroke, pyramidal signs, behavioural changes, and sphincter
disturbance
[9,15]
. This direct neurological involvement can lead to
secondary headaches, but primary headache syndromes (such as
migraine and tension headache) affect 50% of patients with BD
and are responsible for 70% of all headaches in BD
[12]
. However,
headaches in BD patients might be an indication of the neurolo-
gical involvement of BD, but most of those headaches are
benign
[9]
. Sometimes BD could be misdiagnosed as aseptic
meningitis, multiple sclerosis, or primary neoplasm
[9]
. On the
other hand, some studies indicated that although headache is so
Table 1
Demographic descriptive statistics of the sample
BD patients
(n=60)
Control
(n=60)
Total
(n=120) P
Sex, n (%) 0.695
F 18 (30.0) 20 (33.3) 38 (31.7)
M 42 (70.0) 40 (66.7) 82 (68.3)
Age, years, n (%) 0.269
2834 37 (61.7) 31 (51.7) 68 (56.7)
3541 23 (38.3) 29 (48.3) 52 (43.3)
Systolic blood
pressure, mmHg
125.1 121.7 0.007
BD, Behcets disease; F, female; M, male.
Table 2
Frequency of the symptoms in BD patients
Symptoms N(%)
Oral aphtus 60 (100.0)
Genital ulcer 36 (60.0)
Ocular manifestations 33 (55.0)
Arthralgia and arthritis 30 (50.0)
Erythema nodosum 24 (40.0)
Vascular manifestations 24 (40.0)
Pseudo-folliculitis 18 (30.0)
Gastrointestinal manifestations 7 (11.7)
BD, Behcets disease.
Rabah et al. Annals of Medicine & Surgery (2024)
2551
high among BD patients, they do not consider this as a neurolo-
gical involvement sign but only a major complaint of many
patients
[16,17]
.
The characteristics of primary headaches such as migraine, ten-
sion-type and cluster headaches, and secondary headaches related
to ocular involvement, and non-structural headaches have been a
eld of interest in many studies. Meanwhile, the rarity and specic
geographical distribution of BD have resulted in studies with small
sample sizes, and all reached challenging disparities results
[6,7,9,10]
.
We found a higher prevalence of headaches in BD patients like
Borhani et al.
[7]
Volpinari et al.
[18]
, and Kale et al.
[10]
the study,
but less than the percentage found in some other studies, like Kidd
et al.
[12]
, Monastero et al.
[19]
, and Turkish Headache
Epidemiology Study Group
[20]
Saip et al.
[21]
. These differences
may be due to the ethnicity variables.
Tension-type headaches and uveitis-related headaches were
more frequent among patients with BD than the control group,
according to a study by Vinokur et al.
[22]
, and this was similar to
our ndings.
However, uveitis had a signicantly positive correlation with the
frequency of headaches in BD patients
[12,19]
. It differs from another
study
[10]
which found that the most extra parenchymal involve-
ment was venous thrombosis, seizure disorder, and psychiatric
problems/depression or anxiety. Also, Vinokur et al.
[22]
,intheir
results, found that the most common cause of secondary headache
was venous thrombosis. The frequency of non-structural headaches
was one-third of that reported in the study carried out in Turkey
[20]
and we have only recorded two patients suffering from this type of
headache in our study and may be the larger sample size of the
Turkish study had played a role.
Previous studies reported a link between blood pressure and
headache
[23]
. In our study, the mean systolic blood pressure was
signicantly higher in patients with BD. This might be due to the
consumption of prednisolone or atherogenic pathologies of BD.
We did not compare patients with BD to the control group who
had normal systolic blood pressure to make sure that the differ-
ence in blood pressure does not cause dissimilarity in the pre-
valence of headaches among the case and control groups.
Some opinions suggest that steroids may be used in a dose of
more than 30 mg/day for 15 days in the treatment of BD-related
headaches
[24,25]
. The prevalence of headaches was not sig-
nicantly different between prednisolone-received patients and
those who did not consume prednisolone, so the hypothesis that
prednisolone reduces the prevalence of headaches in BD
[10]
was
not conrmed in our study. Therefore, the doses of prednisolone
used for the treatment of BD (7.515 mg/daily) exert a weak effect
on headaches
[10]
. Otherwise, it is well known that headache is a
less common side effect of cyclophosphamide treatment, which
may be due to the elevation of blood pressure which could happen
during the infusion of cyclophosphamide. No data were found
about the prevalence of headaches during cyclophosphamide
infusion
[26,27]
. The rate of using the treatment for headaches in
our study was higher in both groups compared to the previous
studies
[25,26,28]
. The facility of buying analgesics in Syria may
explain these differences. Moreover, in our study, the presence of
a family history of headaches was not signicantly different
between both groups, which is in concordance with some
studies
[28,29]
.
The strengths of the current study were the use of validated
diagnostic criteria for headaches, and it was revealed for the rst
time in our country.
Limitations
To be noted, in this study, we found no specic pattern for
intensity, characteristics, or localization of headaches in BD
patients. Even if it is the rst research that studies the symptoms of
headache among Syrian Behcet disease patients, but it is major
limitation is the small patient sample size, the enrolment of the
study in one referral centre, and the inability to perform MRI and
HLAB 27 to all patients. The rarity of the disease and its under-
diagnosis in Syria may be the factors that induced a small sample
size and were obstacles in the study. However, our ndings
indicated that headache in Syrian BD patients was not sig-
nicantly different from the healthy population.
Table 3
Comparing between BD patients and control in primary
BD patients (n=60), n(%) Control (n=60), n(%) Total (n=120), n(%) P
Primary headache 22 (36.7) 13 (21.7) 35 (29.2) 0.07
Tension headache 11 (18.3) 6 (10.0) 17 (14.2) 0.19
Migraine without aura 6 (10.0) 4 (6.7) 10 (8.3) 0.50
Migraine with aura 3 (5.0) 3 (5.0) 6 (5.0) 0.66
Cluster headache 2 (3.3) 0 2 (1.7) 0.49
BD, Behcets disease.
Table 4
The secondary headache in BD patients
N(%)
Secondary headache 22 (36.7)
Anterior uveitis 17 (28.3)
Posterior uveitis 8 (13.3)
Macular oedema 2 (3.3)
BD, Behcets disease.
Table 5
Comparing between BD patients and control in treatment, and
family history
BD patients
(n=60), n(%)
Control (n=60),
n(%)
Total (n=120),
n(%) P
Treatment 12 (20.3) 6 (10.0) 18 (15.1) 0.20
Family history 10 (16.7) 6 (10.0) 16 (13.3) 0.42
BD, Behcets disease.
Rabah et al. Annals of Medicine & Surgery (2024) Annals of Medicine & Surgery
2552
Conclusion
Headaches may be an early sign of BD neurological involvement.
Headache, especially tension-type headache, is more frequent in
BD patients. neuro-Behcets (NBD) must be meticulously investi-
gated in patients with BD who presented with headache.
However, further higher sample size or multicenter researches are
highly recommended for a better understanding of neurological
symptoms especially headaches among Syrian BD patients.
Ethical approval
This study was approved by the Ethical Committee of the Faculty
of Medicine, Syrian Private University( QS;32109,2019). We
conrm that all methods employed in this study were carried out
in accordance with the relevant guidelines and regulations. We
have adhered to ethical standards and followed the appropriate
procedures to ensure the integrity and validity of our research.
Consent
Written informed consent was obtained from the patients for this
research publication. A copy of the written consent is available
for review by the editor-in-chief of this journal upon request.
Sources of funding
Not applicable.
Author contribution
All the authors contributed to the study concept and design. K.R.,
N.R. and M.K.: clinically approached the patients. K R., N.R.
and H.D. retrospectively checked the results. H.D. conducted the
statistical analysis K.R. N.R. and G.H. drafted the manuscript.
M.D. revised the manuscript for scientic accuracy. All the
authors revised the nal draft of the manuscript and approved it
for publication.
Conicts of interest disclosure
The authors deny any conicts of interest in regard to the
current study.
Research registration unique identifying number
(UIN)
1. Name of the registry: Maysoun Kudsi.
2. Unique Identifying number or regiistration ID: research
registry9449.
3. Hyperlink to your specic registration (must be publicly
accessible and will be checked): https://www.researchregis
try.com/browse-theregistry#home/registrationdetails/64e8aa
45f4acf50029ff470c/.
Guarantor
Maysoun Kudsi.
Availability of data and material
The datasets, that was used and analyzed during the current
study, are available from the corresponding author on reasonable
request.
Provenance and peer review
Not commissioned, externally peer-reviewed.
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Background Behçet's disease is a multisystem autoimmune disorder of unknown origin, characterized by frequent oral and genital ulcerations, ocular and cutaneous lesions, arthritis, and it may also involve the gastrointestinal, and central nervous system. In this paper we discuss a case of Behcet syndrome presenting with isolated oral aphthous initially and spotlight on the clinical features and treatment. Case report A case of a 32-year-old male with repetitive oral aphthous on the tongue and the inner cheeks for four days with a history of frequent recurrence. The patient responded to topical triamcinolone acetonide and 5–10 mg/day prednisone. Three weeks later, there was a recurrence of oral aphthous, genitalia lesions, and blurred vision with left eye redness. A uveitis diagnosis was made, and the patient was placed on 75mg (1mg/kg) prednisone with a resultant response. There was another recurrence with all the previous symptoms, so the decision with Dopson treatment was made. The patient was placed on Dapsone, resulting in complete resolution of the lesions with no reproduction within and after six months of several recall visits. The diagnosis of Bechet syndrome was made. Discussion Aphthous ulcers may be seen in a variety of disorders, while recurrent and persistent >6 weeks, individual non-healing ulcers require a review of the diagnosis. Behcet disease usually appears 2–3 years after oral and genital ulcers, but oral aphthae may be its first manifestation in 10–20% of cases. AS oral ulcers are among the common complaints of the polyclinics-patients, it is recommended to educate the health professionals to diagnose these unusual cases in the first instance, thus avoiding underdiagnosed and the time management to reduce complications and improve the quality of life in these patients. Conclusion A case of Behçet's disease in a 32-year-old male who presented initially with oral mucosa and later caused uveitis and genitalia aphthae.
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Purpose of review: Our objective is to describe the various neurologic manifestations of Behcet's syndrome with special attention to headache syndromes. Recent findings: Most recent studies have focused on data collection to better characterize the relatively rare disorder of neuro-Behcets (NBD). In patients with Behcet's disease (BD) who are experiencing headache, most are primary headache disorders, specifically tension type or migraines. Worsening headache can, however, be a sign of increased Behcets disease activity. Behcet's disease is a chronic, systemic, inflammatory disorder of unknown etiology. The prevalence of BD is most notable in countries along the ancient Silk Road, particularly the Middle East. BD occasionally has neurologic manifestations, further categorized into parenchymal and non-parenchymal syndromes. Headache is the most frequently reported neurological symptom of BD. Primary headaches are the most common headache type among patients with BD with comorbid headache disorders with debate regarding whether migraine or tension-type headache is most prevalent. Of the secondary causes of headache in BD, cerebral venous thrombosis is the most common.
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Behçet's syndrome (BS) is a systemic vasculitis characterized by a relapsing and remitting course. It can involve the skin, mucosa, joints, vessels (arteries and/or veins), eyes, and nervous and gastrointestinal systems, and so is referred to as a syndrome rather than as a unique and nosologically distinct condition. These involvements may present alone or co-exist in the same patient. Although all the possible combinations of the above-mentioned manifestations may occur, clusters of commonly co-existing involvements (also referred to as 'disease phenotypes') have been suggested, namely 'mucocutaneous and articular', 'peripheral vascular and extra-parenchymal neurological' and 'parenchymal neurological and ocular' phenotypes have been described. Patient-specific demographic and genetic features have been described as positively or negatively associated with specific disease phenotypes. This review will focus on the different clinical features of Behçet's syndrome, summarizing current evidence on the distinct disease manifestations as well as the major phenotypes.
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Background: Behcet's disease involves several systems in the body. Neurological involvement is identified by different symptoms. Headache is one of the common complaints of patients with Behcet's disease. It might be a part of neurological involvement or may arise independently in the course of disease. Studies with small sample size have resulted in various findings in this field. Since the prevalence of Behcet's disease is relatively high in Iran, this study was carried out to compare the features of headache between an acceptable number of patients with this rare disease and a control group. Methods: The current case-control study was performed to compare the features of headache between 312 patients with definite Behcet's disease who referred to a Behcet's clinic and healthy individuals. Patients with Behcet's disease were randomly selected. Controls were matched for age and sex. They were personally examined and interviewed meticulously using a questionnaire that met the standards of the International Headache Society classification for different types of headache. Results: The incidence of headache in the case and control groups was 28.3 % (n = 120) and 18.6 % (n = 59), respectively (p < 0.05; OR 2.73). Tension-type headache was observed in 12.2 % (n = 38) of cases which was significantly higher than control group (n = 6.3 %) (p = 0.011; OR 2.05). The most frequent type of headache in the case group was tension-type headache (12.2 %). In the control group, however, migraine without aura was the most common type (9.1 %). A correlation between ophthalmological involvement and headache was observed in 11 patients in the case group. In addition, a significantly higher systolic blood pressure was found in the case group compared to the controls (125.1 vs. 121.7 mmHg; p = 0.007). There was no major correlation between prednisolone consumption in patients with Behcet's disease and the type and frequency of headache. Conclusions: Headache, especially tension-type headache, is more common in patients with Behcet's disease. This might be the result of specific types of uveitis-related and non-structural headaches seen in Behcet's disease.
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Introduction Strengthening The Reporting Of Cohort Studies in Surgery (STROCSS) guidelines were developed in 2017 in order to improve the reporting quality of observational studies in surgery and updated in 2019. In order to maintain relevance and continue upholding good reporting quality among observational studies in surgery, we aimed to update STROCSS 2019 guidelines. Methods A STROCSS 2021 steering group was formed to come up with proposals to update STROCSS 2019 guidelines. An expert panel of researchers assessed these proposals and judged whether they should become part of STROCSS 2021 guidelines or not, through a Delphi consensus exercise. Results 42 people (89%) completed the DELPHI survey and hence participated in the development of STROCSS 2021 guidelines. All items received a score between 7 and 9 by greater than 70% of the participants, indicating a high level of agreement among the DELPHI group members with the proposed changes to all the items. Conclusion We present updated STROCSS 2021 guidelines to ensure ongoing good reporting quality among observational studies in surgery.
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Objective: To assess the efficacy and safety of treatment modalities for major organ involvement of Behçet's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS. Methods: A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included. Results: We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A. Conclusion: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.
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The aims of the present study were to evaluate the prevalence of headache and the frequency of different headache syndromes in patients with Behcet's Disease (BD) without neurological involvement and to investigate the relationship with other clinical, and behavioural variables. Twenty-seven BD patients and 27 control subjects underwent a validated semistructured questionnaire based on the International Headache Society criteria. Levels of anxiety and depression, disease activity, and current medication were collected. Headache occurred in 88.9% of BD patients. There was no difference in the prevalence of the different headache syndromes between BD patients and controls. Only migraine without aura (MwA) was significantly more frequent in BD patients than controls (44.4% vs. 11.1%, respectively, P = 0.013). No relationship was found between MwA and clinical, and behavioural variables. Among headache syndromes, MwA showed the highest frequency in BD. A vascular or neuronal subclinical dysfunction could justify this association. A careful interview for migraine might be included in the diagnostic work-up of BD.