Behçet's Syndrome Patients Research Articles

AB0602 Anti-TNF Treatment for Refractory Vascular Involvement of BehÇEt's Syndrome: A Report of 16 Patients

BackgroundThe optimal management of vascular involvement and especially pulmonary artery involvement (PAI) in patients with Behçet's syndrome (BS) is still a challenge.ObjectivesTo evaluate the efficacy and safety of anti-TNF therapy...

Correlation between vitamin D status in Behçet syndrome and disease activity, severity and patient's quality of life

Objectives: Studies concerning vitamin D status in Behçet syndrome (BS) are scarce and their results are controversial. None of these works analyzed the link between this status and the disease activity, severity and the patient's quality of life.The aim of our work was to analyze the possible association between vitamin D status in BS and the disease activity, severity and the patient's quality of life. Methods: 25(OH)D3 was assessed in 40 BS patients (mean age: 37.5years, sex ratio: 1.85) by electrochemiluminescence.

Henoch Schönlein purpura at diagnosis in 3 adult patients: Acute onset with peripheral nervous system dysfunction

Objectives: Studies concerning vitamin D status in Behcet syndrome (BS) are scarce and their results are controversial. None of these works analyzed the link between this status and the disease activity, severity and the patient's quality of life.The aim of our work was to analyze the possible association between vitamin D status in BS and the disease activity, severity and the patient's quality of life.Methods: 25(OH)D3was assessed in 40 BS patients (mean age: 37.5 years, sex ratio: 1.85) by electrochemiluminescence. Its levelwas then correlated to theBS activity, severity and patient's quality of life. A BS expert evaluated patients as having active or inactive disease. BS activity was also assessed meaning the BDCAF score (Behcet's Disease Current Activity Form). The disease severity was appreciated with the severity index. The patient's quality of life was evaluated by a specific score the BDQoL (Behcet's disease quality of life). Results: At BS diagnosis, patients were aged 28.83 +/− 11 years on average. The mean disease duration was at 8.75 years +/− 10 years. 25(0H)D3was at 14.64 ng/ml (3– 44.29 ng/ml) and nearly 1/3 of patients (35%) had a vitamin D deficiency.BD expert estimated that the disease was active in 40% of the cases.Themean BDCAF score was at 4.86 (0–11). This score correlated well with the expert's opinion. The mean index severity was 4.7 +/− 1.86 (2–11) and the mean BDQoL was 7.78 +/− 6.55 (0–30). Correlation analysis didn't showany link between vitamin D status in BD patient with the disease duration, activity and severity and with the patient's quality of life. Conclusions: Our work didn't showany association between vitaminD status in BDpatientswith the disease characteristics (duration, activity, severity) and patient's quality of life. It can be due to the lack of specificity of vitamin D role in immune diseases but we think that further studies with larger samples are needed.

AB0454 Suicidal ideation among patients with behcet’s syndrome

BackgroundAn increased frequency of depression has been reported in Behcet’s syndrome (BS) (1). While an increased suicidal ideation has been reported in other chronic rheumatologic conditions (2), this has been...

FRI0227 Characteristics, treatment and outcome of GI involvement in behcet syndrome: Experience in a dedicated center

BackgroundGastrointestinal involvement can be a severe complication of Behcet’s syndrome (BS) resulting in perforation and massive bleeding. Controlled data regarding treatment is lacking and long term prognosis is not well...

OP0211 A Preliminary Investigation of “Treat-to-Target” Criteria Using BSAS, RAPID3 and Physician Global in Behcet’s Syndrome”

BackgroundWe had developed a PRO measure for Behcet Syndrome (BS) BSAS (Behcet Syndrome Activity Scale), for use as an outcome measure in order to determine need for treatment changes, leading...

Behçet's Syndrome in Iranian Azari People

Behçet's Syndrome (BS) is a chronic recurrent multisystemic inflammatory disorder characterized by oral and genital ulcers, ocular inflammation. Behçet's syndrome has a complex genetic etiology. However, epidemiological studies recommend that genetic factors have a significant influence to its pathogenesis, alike to other autoinflammatory disorders. Epidemiological statistics, clinical records and HLA typing were studied in Iranian Azari patients with Behçet's syndrome. This investigation considered HLA associations with BS and HLA with certain clinical characteristics, age and sex in the (Tabriz) Iran which has an ethnically homogeneous population. HLA-A and HLA-B typing was performed in 290 BS patients, conforming to International Study Group criteria and in 300 blood donors, as controls. Patient records were retrospectively reviewed and patients reassessed clinically. HLA-B5, HLA-B35, HLA-51, HLA-B52 and HLA-CW4 presented significantly high frequencies in all patients. No other HLA type was associated. There was a significant HLA link with male sex in BS patients and Mean age (34 +/- 1.1) was determined. We present the frequency and correlation between Iranian Azari patients with Behçet's syndrome and particular HLA antigens. Ninety nine percent had mouth ulceration, 64% genital ulceration, 72% skin lesions and 52% ocular involvement. This study supports HLA-B5, HLA-B35, HLA-51, HLA-B52 and HLA-CW4 immunogenetic predisposition in an ethnically homogeneous (Iranian Azari) population.

Vascular neurobehcet disease:correlation with current disease activity forum and systemic vascular involvement

Behcet's syndrome (BS) is a chronic relapsing vascular inflammatory disease of unknown etiology with high morbidity and mortality. This research aims to study the clinical patterns of CNS disease in a group of patients with BS as well as the frequency and type of the associated radiographic findings suggestive of structural cerebral vascular disease. The findings were studied in relation to disease activity and features of systemic vascular involvement. Forty patients fulfilling the diagnostic criteria of the International Study Group for Behcet's Disease, mean age of 33.56 ± 9.7 years, were enrolled. Patients were subjected to magnetic resonance imaging with conjugate survey of cerebral blood vessels' flow pattern abnormalities by transcranial Doppler study. Thirty healthy controls were included. Behcet's Disease Current Activity Form Score was used. Neuro-Behcet's syndrome (NBS) was diagnosed in 37.5% with headache being the most common (86.6% of cases), pyramidal affection (signs of upper motor neuron lesions/hemiplegia) was reported in 33.3%, attacks of disturbed conscious level in 26.6%, and cranial nerve affection in 6.5%. Of the patients, 66.6% with clinical features of NBS had statistically significant radiographic evidences of cerebrovascular disease (p = 0.01). Patients with NBS had significantly higher disease activity index score (r = 0.69, p = 0.0001). Radiographic findings and flow abnormalities were significantly less in patients on immune suppressants and antiplatelet drugs (p = 0.003, 0.04). BS patients with clinical neurologic disease were found to have radiographic findings suggestive of cerebral vascular disease with high disease activity index score. Drugs like immunosuppressants and oral antiplatelets might retard cerebral vascular disease progression and flow abnormalities, respectively.

The papulopustular lesion/arthritis cluster of Behcet's syndrome also clusters in families

We have previously reported distinct symptom clusters among our patients with Behçet's syndrome (BS). The presence of such clusters suggests that more than one pathogenetic mechanism might be operative in BS. Increases in the frequency of certain clusters in familial BS cases, if present, would further support this notion. To test this hypothesis, we compared the frequency of symptom clusters between familial (group F) and non-familial (group NF) cases of BS. We identified 380 BS patients who had reported a first-degree relative by reviewing 6031 patient charts. We were able to contact 186 (Group F). From the same initial pool, 500 patients were randomly selected. Of those, patients who did not report a family history of BS and who had attended our clinic during the previous 3 months made up group NF (n = 221). Both groups were questioned about their symptoms within the previous 3 months. Data were analysed using factor analysis, cluster analysis and χ2 tests. The make-up of the symptom clusters were very similar for the factor and the cluster analyses. The frequency of papulopustular lesions and joint involvement cluster was significantly higher in group F (39.2 vs. 21.5%, P < 0.001). Furthermore, the same cluster was shared in 5/17 related pairs from group F and in only 5/110 unrelated pairs from group NF [29 vs. 4.5%, P = 0.004; risk ratio (RR) = 6.47, 95% CI 2.15, 18.89]. The papulopustular lesions and arthritis cluster in BS appears to cluster in familial BS as well. This further supports the notion that the pathogenesis of BS may entail several distinct mechanisms resulting in separate phenotype clusters.

Analyses of functional IL10 and TNF-α genotypes in Behçet’s syndrome

We aim to ascertain the possible involvement of functional IL10 and TNF-α promoter polymorphisms on the susceptibility to Behçet's syndrome (BS), to examine whether IL10 and TNF-α genotypes might work synergistically influencing susceptibility to BS. IL10 -1082G/A, -819C/T and -592C/A and TNF -308G/A polymorphisms were analyzed in 102 Turkish patients with BS and 102 healthy subjects by using amplification refractory mutation system-polymerase chain reaction (ARMS-PCR). We have found no significant associations between IL10 -1082G/A, -819C/T, -592C/A, TNF-α -308G/A polymorphisms and BS. Also, no significant correlation was found between IL10 GCC, ACC, ATA haplotypes, GCC(+)/GCC(+), GCC(+)/GCC(-), GCC(-)/GCC(-) genotypes. There was no significant association between combined TNF-α/IL10 genotypes and BS. Our study indicates that functional TNF-α, IL10 genotypes or combined TNF-α, IL10 genotypes do not play a role in BS susceptibility in Turkish BS patients.

Low-dose natural human interferon-α lozenges in the treatment of Behçet's syndrome

There had been evidence that low-dose local IFN could be beneficial in the management of recurrent oral ulcers (OUs). We investigated the efficacy and collected initial data on the safety of low-dose natural human IFN-alpha administered by the oral mucosal route in Behçet's syndrome (BS) in a placebo controlled, double blind study. Eighty-four (59 males and 25 females) patients with BS with mainly skin mucosa disease and a history of recurrent OU for > or = 1 year were studied. When they had at least two OUs with a total diameter of > or = 4 mm, they were randomly allocated to (i) 2000 IFN-alpha IU/day, (ii) 1000 IFN-alpha IU/day and (iii) placebo groups. Subjects were monitored weekly over an initial 4 weeks and bi-weekly for an additional 8 weeks of treatment. OU were counted and measured at each study visit. The primary efficacy end point was the difference in the total ulcer burden at Week 0 compared with that at Week 12. Out of the 84 patients enrolled, 72 completed the trial. There were no statistically significant differences between the treatment arms in terms of the primary endpoint. Low-dose natural human IFN-alpha did not have beneficial effects on reducing the total ulcer burden among BS patients from Turkey. The study also showed that counting the number of ulcers rather than measuring the size would be adequate in future studies. ClinicalTrials.gov, NCT00483184, http://www.clinicaltrials.gov/ct2/results?term=NCT00483184.

Genetic susceptibility to Behçet’s syndrome is associated with NRAMP1 (SLC11A1) polymorphism in Turkish patients

Natural resistance associated macrophage protein 1 (NRAMP1), also named as solute carrier family 11 member A1 gene (SLC11A1), has multiple pleiotropic effects on macrophage activation pathways such as up-regulation of the CXC chemokine KC, tumor necrosis factor alpha (TNF-alpha), interleukin-1 b (IL-1 b), inducible nitric oxide syntase (iNOS), and major histocompatibility complex (MHC) class II expression. Since NRAMP1 plays a role in the up-regulation of the TNF-alpha, iNOS and MHC expression, it may also be a candidate gene for Behçet's syndrome (BS). We analyzed the association of NRAMP1 polymorphisms [(GT)( n ), INT4, 3'UTR and D543N] in 102 Turkish patients with BS and 102 healthy subjects by using amplification refractory mutation system-polymerase chain reaction (ARMS-PCR). We found a significant association between BS and NRAMP1 INT4 G/C allele frequency (p = 0.004, OR = 1.88, 95% CI = 1.21-2.93). However, there were no significant differences in the distribution of allele frequencies of NRAMP1 (GT)( n ), 3'UTR, D543N polymorphisms between BS patients and healthy controls. There was also no correlation between NRAMP1 polymorphisms and clinical manifestations of BS. Our study suggests that NRAMP1 may be one of the plausible candidate genes for BS. However, it is likely that INT4 polymorphism is not disease-specific and seems to be common to immune-mediated diseases.

Increased Enthesopathy Among Behcet's Syndrome Patients With Acne and Arthritis

Increased Enthesopathy Among Behcet's Syndrome Patients With Acne and Arthritis

Purified protein derivative reaction is not augmented in Behcet's syndrome patients

Purified protein derivative reaction is not augmented in Behcet's syndrome patients

Prevalence of angina, myocardial infarction and intermittent claudication assessed by Rose Questionnaire among patients with Behcet's syndrome

To determine the risk of clinical cardiovascular disease in middle-aged patients with Behcet's syndrome (BS) compared with gender-matched non-BS subjects. The prevalence of angina, myocardial infarction (MI), doctor diagnosed ischaemic heart disease (IHD) and intermittent claudication were sought by the Rose Angina Questionnaire in 225 (141 M/84 F) BS patients (mean age: 52 +/- 8) with BS and 117 (74 M/43 F) controls (mean age: 50 +/- 5). Information on atherosclerotic risk factors was also collected. The prevalence of angina, MI and doctor-diagnosed IHD were not different between BS patients and non-BS controls in the whole study population and when males and females were separately analysed. Angina tended to be more common among females compared with males among both patients and controls. Intermittent claudication was found to be significantly more common among BS patients, especially in males with venous disease. The findings in this cross-sectional clinical study are in line with previous observations not indicating accelerated atherosclerosis in BS. Intermittent claudication might not be a suitable tool for the detection of peripheral atherosclerotic disease especially among BS patients having venous disease.

Neuro-behçet’s syndrome: Differential diagnosis and management

Behçet's syndrome (BS) is a multisystem disease of unknown etiology, characterized by chronic relapsing cardinal symptoms of orogenital ulcers, uveitis, and different skin lesions. Its major pathologic feature is vasculitis. Neuro-Behçet's syndrome (NBS) is defined as a constellation of neurologic manifestations with characteristic neuropathologic findings, usually confirmed by ancillary investigations, in patients who meet the diagnostic criteria for BS. Neurologic manifestations of the syndrome are more common in male patients and have been reported to occur in anywhere from 5% to 50% of BS patients, depending on their geographical region. NBS primarily affects the central nervous system and includes parenchymal and nonparenchymal involvement. Peripheral neuropathy and myopathy are rare. Immunosuppression is widely used for treatment.

Headache in Behçet's Syndrome

To study the frequencies and characteristics of different headache types seen in patients with Behçet's syndrome (BS) in a large cohort of patients. Patients with BS may present with different neurological problems, related either directly or indirectly to the disease, with headache being the most common neurological symptom seen in this syndrome, as well as independent from neurologic involvement. This study was carried out at the multidisciplinary Behçet outpatient clinic of the Behçet's Syndrome Research Center. Every fifth admitted patient who had fulfilled the International Study Group for Behçet's Disease classification criteria was recruited for this study. Each patient was interviewed by one of the examining neurologists through a semistructured questionnaire. In case of the presence of more than one headache type, the best defined one was diagnosed and evaluated. All patients received a complete neurological and physical examination. A chi(2) test was used to evaluate the differences between frequencies. One sample t-test was used to compare means. The prevalence rates of primary headaches were compared with results of the Turkish Headache Epidemiology study by using goodness-of-fit test. Two hundred and twenty-eight patients were studied. Headache was reported in 66.2% (151/228) of the study cohort. Primary headaches were seen in 38.6% of the patient population, which corresponds to 58% of BS patients with any type of headache. Tension-type headache (TTHA) and migraine were seen in 23.6% and 14.9% of the whole study cohort, respectively. In 5.2% of the study cohort, the headache was associated with neurological involvement and in 3.9% to uveal inflammation. A predominantly frontal, bilateral paroxysmal throbbing pain of moderate severity was reported in 18.4% of the study cohort who did not fulfill the criteria of the International Headache Society (IHS) for any of the primary headaches. It was commonly associated with exacerbations of the mucocutaneous symptoms of the syndrome. The neurological examination was normal in all of these patients. This type of headache was categorized as the nonstructural headache of Behçet. Headache is the most common neurological symptom seen in BS, both in patients with and without neurological involvement. The results of our study reveal that headache is seen in a majority of patients with neurological involvement due to BS and in a minority with uveal inflammation. The prevalence of migraine and TTHA are close to the population in general, but a nonstructural migrainous headache, which is commonly associated with exacerbations with some of the systemic symptoms of the syndrome, is noteworthy in patients with BS. This form of headache is not specific for this disorder, but may be explained by a vascular headache triggered by the immunomediated disease activity in susceptible individuals.

Serum Levels of Soluble P‐Selectin Are Increased and Associated With Disease Activity in Patients With Behçet′s Syndrome

Behçet's syndrome (BS) is a relapsing, chronic, inflammatory disease characterized by endothelial dysfunction, atherothromboembogenesis, and leukocytoclastic vasculitis with complex immunologic molecular interactions. Generalized derangements of the lymphocyte and neutrophil populations, activated monocytes, and increased PMNLs motility with upregulated cell surface molecules such as ICAM-1, VCAM-1, and E-selectin, which are found on the endothelial cells, leukocytes, and platelets, have all been demonstrated during the course of BS. Our aim is to investigate the association of serum concentrations of soluble P-selectin in patients with BS, and to evaluate whether disease activity has an effect on their blood levels. This multicenter study included 31 patients with BS (15 men and 16 women) and 20 age- and sex-matched healthy control volunteers (11 men and nine women). Neutrophil count, erythrocyte sedimentation rate, and acute-phase reactants as well as soluble P-selectin levels were determined. The mean age and sex distributions were similar (P > .05) between BS patients (35 years) and control volunteers (36 years). Serum levels of soluble P-selectin in patients with BS (399 ± 72 ng/mL) were significantly (P < .001) higher when compared with control subjects (164 ± 40 ng/mL). In addition, active BS patients (453 ± 37 ng/mL) had significantly (P < .001) elevated levels of soluble P-selectin than those in inactive period (341 ± 52 ng/mL). This study clearly demonstrated that serum soluble P-selectin levels are increased in BS patients when compared with control subjects, suggesting a modulator role for soluble P-selectin during the course of platelet activation and therefore, atherothrombogenesis formation in BS, especially in active disease.

A reappraisal of amyloidosis in Behçet's syndrome.

To evaluate the clinical features and outcome of patients with Behçet's syndrome (BS) and amyloidosis and to assess the associated risk factors. A chart review was done to determine the frequency of amyloidosis in BS among 4000 patients. Data from 14 BS patients with amyloidosis were compared with data obtained from 718 patients with BS without amyloidosis. Multiple stepwise logistic regression analysis was used to assess the risk factors. All patients with amyloidosis presented with the nephrotic syndrome or significant proteinuria. The mean time to the onset of amyloidosis was 8.1 yr (range 3-15 yr). The mean duration of follow-up after amyloidosis was 3.4 yr (range 1-11 yr). Seven out of 14 patients were alive at the time of the evaluation. Peripheral and pulmonary arterial involvement and arthritis were associated with amyloidosis (P<0.05). Amyloidosis in BS is rare and has a 50% mortality rate at 3.4 yr (range 1-11 yr). Peripheral and pulmonary arterial involvement and arthritis seem to be the strongest predictors of amyloidosis in BS.

Wound healing in Behçet's syndrome.

Because there is an increased inflammatory response to trauma, particularly of the skin, in patients with Behçet's syndrome (BS), an alteration in wound healing in BS is expected. The aim of this study was to investigate the healing features of punch biopsy wounds in BS and acne vulgaris (AV) patients used as controls. Full-thickness skin punch biopsies (4 mm) were taken from the hairless sites of the non-dominant forearms in 20 BS and 20 AV patients. Each patient was examined on days 1, 2, 3, 4, 5, 8, and 10, and the biopsy wound area and induration were marked on sterile glass slides. Other inflammatory changes, such as suppuration and pain, were also recorded. No antiseptic solutions or ointment, except saline, were used. The wound area healed similarly in both groups (p > 0.05). Thirteen (65%) BS patients had erythematous haloes around the wound on the first day. The number of patients presenting inflammatory changes reached 18 (90%) on the second day in the BS group. The area of erythema around the wound in BS patients was significantly greater than that in AV patients (5; 25%). It gradually decreased day by day. Purulent changes were observed in four BS patients on day 1, and induration around the biopsy wound in six patients on day 2. Only one patient with AV had suppuration, which appeared on the second day, while no AV patient displayed induration. Biopsy-induced trauma may cause increased inflammation in BS, but wound healing is not altered.