scholarly journals Phenotype heterogeneity in Late-onset Behçet’s syndrome: A cohort from a referral center in China

2020 ◽  
Author(s):  
Jun Zou ◽  
Jian-feng Luo ◽  
Dan Luo ◽  
Yan Shen ◽  
Jian-long Guan
Keyword(s):  
Cluster Analysis ◽  
Late Onset ◽  
Disease Onset ◽  
Ocular Involvement ◽  
Behçet’S Syndrome ◽  
Intestinal Lesion ◽  
Clinical Variables ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Objectives To elaluate the demographic, clinical and major organ involvement of patients with late-onset Behçet’s syndrome (BS) in a tertiary centre in China.Methods It was a cross-sectional study of consecutive BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020, we identified 152 late-onset BS patients diagnosed after age of 40 years. We compared clinical variables between patients with disease onset age before and after 40 years. The relative risks (RRs) of clinical variables were calculated between two age groups. Moreover, a hierarchical cluster analysis was conducted according to twenty-nine variables to determine homogeneous subgroups of patients.Results Late-onset BS patients had a higher incidence of intestinal ulcer (late-onset : early-onset, RR 1.47), but a lower incidence of ocular involvement (RR 0.54) and folliculitis (RR 0.46). Female was associated with genital ulcer, erythema nodosum, and arthritis. Four clusters (C1–C4) were formed. C1 (n = 71) the largest cluster, defined as mucocutaneous group. C2 (n = 20), the arthritis group, C3 (n = 39), the gastrointestinal type, all patients presented with intestinal lesion, and five cases with esophageal ulcer. In C4 (n = 22), showing a mixture of uveitis and vascular group, fifteen patients presented with uveitis and eight had vascular lesion, and one cases had central nervous system lesions.Conclusion This is the first cluster analysis to be carried out in a cohort of late-onset BS patients in China. The significantly lower prevalence of folliculitis in late onset BS and the overall milder disease course suggest that testosterone status may influence disease activity.

scholarly journals Discordance between patient and physician global assessment of disease activity in Behçet’s syndrome: a multicenter study cohort

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Alberto Floris ◽  
◽  
Gerard Espinosa ◽  
Luisa Serpa Pinto ◽  
Nikolaos Kougkas ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Inactive Disease ◽  
Ocular Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Patient Reported ◽  
Behcet’S Syndrome ◽  
Assessment Of Disease Activity ◽  
Behcet's Syndrome

Abstract Background To compare the patients’ and physician’s global assessment of disease activity in Behçet’s syndrome (BS) and investigate the frequency, magnitude, and determinants of potential discordance. Methods A total of 226 adult BS patients with a median (IQR) age of 46.9 (35.6–55.2) years were enrolled across Italy, Greece, Portugal, and Spain. Demographic, clinical, and therapeutic variables, as well as the patient reported outcomes, were collected at the recruitment visit. The physical (PCS) and mental (MCS) component summary scores of the Short Form Questionnaire 36 (SF-36) and the Behçet’s syndrome Overall Damage Index (BODI) were calculated. Disease activity was assessed by the patients’ (PtGA) and physician’s global assessment (PGA) in a 10-cm visual analog scale, as well as the Behçet Disease Current Activity Form (BDCAF). Discordance (∆) was calculated by subtracting the PGA from the PtGA and defined as positive (PtGA>PGA) and negative (PtGA<PGA) discordance using both a more stringent (∆ = ±2) and a less stringent (∆ = ±1) cutoff. Univariate and multivariate logistic regressions were performed. Results Median PtGA and PGA scores were 2.0 (0.3–5.0) and 1.0 (0.0–3.0) cm, respectively. The discordance prevalence varied (from 29.6 to 55.3%) according to the cutoff applied, and the majority (> 80%) of disagreements were due to patients rating higher their disease activity. Higher values of BDCAF were associated to increased rate of positive discordance. When BDCAF = 0, the median (IQR) values of PtGA and PGA were 0.2 (0–2) and 0 (0–1), respectively. PCS (adjusted odds ratio (adjOR) 0.96 per unit, 95% CI 0.93–0.98, p = 0.006) and MCS (adjOR 0.96 per unit, 95% CI 0.93–0.99, p = 0.003) were independently associated with positive discordance using both cutoffs. Active ocular involvement emerged as a potential determinant of negative discordance (adjOR 5.88, 95% CI 1.48–23.30, p = 0.012). Conclusions PtGA and PGA should be considered as complementary measures in BS, as patients and physicians may be influenced by different factors when assessing active disease manifestations. Particularly, PtGA may be a useful tool in the assessment of BS disease activity, as it carries a low risk to misclassify an inactive disease, and may allow to capture aspects of the patient’s health that negatively affect his well-being and the treatment.

scholarly journals AB0532 CORRELATION WITH THE FREQUENCY OF DISEASE RELAPSES DURING THE FIRST 3 YEARS FROM THE DIAGNOSIS AND DISEASE OUTCOMES IN BEHCET’S SYNDROME

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1563.2-1563
Author(s):  
R. Talarico ◽  
A. Figliomeni ◽  
L. Mione ◽  
A. Parma ◽  
E. Cioffi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Disease Onset ◽  
Disease Outcome ◽  
Vascular Involvement ◽  
Joint Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Disease Outcomes ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Background:Beside the organ involvement, a number of demographic factors could considerably influence the long-term and short-term outcomes of Behçet’s syndrome (BS): age at disease onset, duration of disease, gender and sex. Younger men patients are more suitable to have a more severe disease, due to an increased frequency both of morbidity and mortality, related to ocular,vascular and neurological involvementObjectives:The primary aims of the study were to evaluate disease activity in a cohort of BS patients consecutively followed in a BS clinic of a tertiary centre and to explore whether there is a correlation between frequency of relapses in the first 3 years of diseases and disease outcomes.Methods:One-hundred and sixty-five patients (91 males and 74 females; mean age 39±9 years, mean disease duration 9±5) with a diagnosis of BS according to the ISG criteria were studied. Disease activity has been evaluated by BDCAF and patients were also categorized in major or minor involvement of BS according or not to the presence of ocular, neurological and vascular involvement in the course of disease The numbers of relapses in the first 3 years from diagnosis were correlated with disease outcome and damage.Results:At time of the evaluation, 47% of BS patients presented an active disease; 69 patients presented muco-cutaneous involvement, 39 ocular disease, 21 joint involvement, 12 neurological impairment and 9 gastro-enteric involvement. Seventy-nine percent of patients presented in the course of the disease a severe BS involvement and the majority was represented by patients characterised by a more frequent relapse in the first 3 years of disease (M/F: 65/48, mean age 43±3 years). Those patients who experienced a more higher number of relapse in the first 3 years compared to the others were also characterised by poor disease outcomes and worse prognosis over time and this correlation was independent by the therapies taken.Conclusion:The high frequency of relapses during the first three years from diagnosis may be considered an important prognostic factor for disease outcome in BS patients, therefore could be taken into account as a useful element to tailor the management, not only according to the type and severity of symptoms and epidemiological factors.Acknowledgments:noneDisclosure of Interests:None declared

scholarly journals Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Jun Zou ◽  
Jian-feng Luo ◽  
Yan Shen ◽  
Jian-fei Cai ◽  
Jian-long Guan
Keyword(s):  
Central Nervous System ◽  
Cluster Analysis ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
System Involvement ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Introduction Behcet’s syndrome (BS) is a complex, heterogeneous disorder. However, classification of its subgroups is still debated. The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements. Methods This was a cross-sectional study of BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020. We calculated relative risks (RRs) of clinical variables according to sex. Moreover, we conducted a hierarchical cluster analysis applied according to eighteen variables to determine subgroups of patients. Results A total of 860 BS patients were included. Male sex was associated with ocular involvement (RR 2.32, 95% CI 1.67, 3.22, P < 0.0001), vascular involvement (RR 2.00, 95% CI 1.23, 3.23, P = 0.004), cardiac lesion (RR 5.46, 95% CI 2.33, 12.77, P < 0.0001), and central nervous system involvement (RR 2.95, 95% CI 1.07, 6.78, P = 0.007) and was negatively associated with genital ulcers (RR 0.84, 95% CI 0.79, 0.91, P < 0.0001). Five clusters (C1–C5) were observed. C1 (n = 307) showed the skin and mucosa type. In C2 (n = 124), all had articular involvement, barely having major organ involvement except for 18 cases with intestinal lesions. In C3 (n = 156), the gastrointestinal type, 144 patients presented with intestinal involvement, and 36 patients with esophageal ulcers. In C4 (n = 142), all subjects presented with uveitis. C5 (n = 131) consisted of 44 patients with cardiac lesions, 58 with vascular involvement, and 26 cases having central nervous system involvement. Conclusion Our analysis confirmed sex differences in phenotypes of BS. Cluster analysis identified gastrointestinal, uveitis, and cardiovascular involvement cluster separately in different subsets, which represents the most commonly involved organs. Further research is required to replicate and clarify the patterns of phenotype in BS.

scholarly journals Side Effects of Azathioprine in Patients with Ocular Behçet’s Syndrome

2020 ◽  
Author(s):  
Maryam Masoumi ◽  
Fereydoun Davatchi ◽  
Farhad Shahram ◽  
Maassoumeh Akhlaghi ◽  
Seyedeh Tahereh Faezi ◽  
...  
Keyword(s):  
Side Effects ◽  
Adverse Events ◽  
Skin Lesions ◽  
Skin Tumor ◽  
Ocular Involvement ◽  
Behçet’S Syndrome ◽  
Cross Sectional ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract BackgroundBehçet’s syndrome (BS) is a multifactorial, polygenic, autoinflammatory vasculitis characterized by recurrent oral and genital ulcers, uveitis, skin lesions, and arthritis. Azathioprine has been well established as an effective therapy among other immunosuppressive drugs; however, concerns remain about its safety. This study was conducted to determine the types and prevalence of adverse events related with azathioprine in patients with ocular BS.MethodsWe carried out a cross-sectional study of 165 patients with a confirmed diagnosis of BS who had ocular involvement. Data were collected retrospectively on disease-related characteristics and events including severity, recurrence, relapse, recovery and flare-up, as well as on azathioprine dosage and the duration of use.ResultsOf 165 enrolled patients, 103 (62.43%) were male and 62 (37.57%) were female. Adverse events of treatment with azathioprine occurred in 11 patients (6.67%). These side effects included elevation of liver enzymes in 4 patients (2.43%), hypersensitivity reaction in 2 patients (1.21%), leukopenia in 2 patients (1.21%), nausea in 1 patient (0.61%), skin tumor in 1 patient (0.61%), and concomitant pancreatitis and hepatitis in 1 patient (0.61%). Six of these patients discontinued treatment, and 5 of them tapered the dose. In addition, we observed severe leukopenia in 2 patients.ConclusionAdministration of azathioprine with due attention to the signs, symptoms, and severity of the disease, results in a reduced rate of adverse events and resolution of the ocular involvement in patients with BS.

Ocular Involvement of Behçet’s Syndrome: a Comprehensive Review

2014 ◽  
Vol 49 (3) ◽  
pp. 298-306 ◽  
Author(s):  
Yilmaz Ozyazgan ◽  
Didar Ucar ◽  
Gulen Hatemi ◽  
Yusuf Yazici
Keyword(s):  
Ocular Involvement ◽  
Behçet’S Syndrome ◽  
Comprehensive Review ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

scholarly journals SAT0273 PROGNOSTIC FACTORS FOR POOR OUTCOME IN BEHÇET’S SYNDROME WITH NEUROLOGICAL INVOLVEMENT: RESULTS FROM A CLINICAL LONG-TERM FOLLOW-UP OF A SINGLE CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1080.2-1081
Author(s):  
D. Sahin Eroglu ◽  
M. Torgutalp ◽  
S. Sezer ◽  
A. Boyvat ◽  
C. Yucesan ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Ocular Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Factors Associated ◽  
Poor Outcome ◽  
Imaging Characteristics ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Background:Behçet’s Syndrome (BS) is a vasculitis of unknown cause. Neurological disease is a type of its serious involvement and causes morbidity and mortality.Objectives:In this study, we aimed to describe the clinical characteristics of neuro-Behçet Syndrome (NBS) and to define prognostic factors that were associated with poor outcome.Methods:Among 2033 patients with BS, we performed a retrospective analysis of 94 patients (52.1% male; mean age 36.1 (11.9) years), who fulfilled the ISG-1990 for BS. We divided patients into two subgroups, either parenchymal (p-NBS) or non-parenchymal (np-NBS). Clinical, laboratory, and imaging characteristics of the patients were defined. We described the poor outcome as a modified Rankin score (mRS) >= 3 at last follow-up and/or death, and assessed the predictor factors associated such kind of outcome.Results:In total, 52 (55.3%) patients presented with p-NBS, of whom 15 (28.8%) had progressive course, and 42 (44.7%) presented with np-NBS (Table.1). Ocular involvement was more common in p-NBS than np-NBS (59.6% vs. 39.1% respectively, p=0.04), whereas vascular involvement excluding sinus vein thrombosis was more frequent in patients with np-NBS (15.4% vs. 50.0%, respectively, p=0.001). Headache and papilledema were frequently observed in np-NBS (p<0.001), whereas paresis, sensory symptoms, pyramidal and cerebellar signs were significantly common in p-NB (<0.001). Twenty-five patients (26.6%) experienced at least one relapse. After a median of 54 (IQR 79.4) months, 11 patients (11.7%) had disabling sequela and a total of 5 (5.3%) deaths occurred. Characteristics of patients with and without poor outcome are presented in table 2. In multivariable logistic regression analysis, factors associated with poor outcome were initial mRS (OR 24.2 (95% CI 3.16 – 108.67)) and age >= 40 at NBS diagnosis (OR 4.59 (95% CI 1.02 – 20.69)), meanwhile, headache at presentation was associated with a lower risk for poor outcome. (OR 0.22 (95% CI 0.05 – 0.91)).Conclusion:Neurologic involvement is a detrimental manifestation of BS and causes disability, even death. Patients who have an initial disabling presentation in advanced age are more likely to have poor prognosis. Treatment intensification in this subpopulation might be considered.References:NoneDisclosure of Interests:None declared

scholarly journals Side Effects of Azathioprine in Patients with Ocular Behçet’s syndrome

2020 ◽  
Author(s):  
Maryam Masoumi ◽  
Fereydoun Davatchi ◽  
Farhad Shahram ◽  
Maassoumeh Akhlaghi ◽  
Seyedeh Tahereh Faezi ◽  
...  
Keyword(s):  
Side Effects ◽  
Adverse Events ◽  
Skin Lesions ◽  
Skin Tumor ◽  
Ocular Involvement ◽  
Behçet’S Syndrome ◽  
Cross Sectional ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract BackgroundBehçet’s syndrome (BS) is a multifactorial, polygenic, autoinflammatory vasculitis characterized by recurrent oral and genital ulcers, uveitis, skin lesions, and arthritis. Azathioprine has been well established as an effective therapy among other immunosuppressive drugs; however, concerns remain about its safety. This study was conducted to determine the types and prevalence of adverse events related with azathioprine in patients with ocular BS.MethodsWe carried out a cross-sectional study of 165 patients with a confirmed diagnosis of BS who had ocular involvement. Data were collected retrospectively on disease-related characteristics and events including severity, recurrence, relapse, recovery and flare-up, as well as on azathioprine dosage and the duration of use.ResultsOf 165 enrolled patients, 103 (62.43%) were male and 62 (37.57%) were female. Adverse events of treatment with azathioprine occurred in 11 patients (6.67%). These side effects included elevation of liver enzymes in 4 patients (2.43%), hypersensitivity reaction in 2 patients (1.21%), leukopenia in 2 patients (1.21%), nausea in 1 patient (0.61%), skin tumor in 1 patient (0.61%), and concomitant pancreatitis and hepatitis in 1 patient (0.61%). Six of these patients discontinued treatment, and 5 of them tapered the dose. In addition, we observed severe leukopenia in 2 patients.ConclusionAdministration of azathioprine with due attention to the signs, symptoms, and severity of the disease, results in a reduced rate of adverse events and resolution of the ocular involvement in patients with BS.

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