Search

To evaluate the demographics, clinical aspects, and major organ involvement of patients with late-onset Behçet's syndrome (BS) in a tertiary center in China.We conducted a cross-sectional study of consecutive BS patients in Huadong Hospital of Fudan University from September 2012 to January 2020. We compared clinical variables between patients with disease onset before and after 40 years of age. The relative risks (RRs) of clinical variables were calculated between the two age groups. Moreover, a hierarchical cluster analysis was conducted according to 29 variables to determine homogeneous subgroups in patients with late-onset BS.We enrolled 152 late-onset BS patients, with a median age at onset of 47 years (interquartile range, IQR: 43-52 years). There is a higher prevalence of intestinal ulcers in late-onset BS than in early-onset BS (RR 1.47), but a lower prevalence of ocular involvements (RR 0.54) and folliculitis (RR 0.46). Female sex was associated with genital ulcers, erythema nodosum, and arthritis. Four clusters (C1-C4) were formed. C1 (n = 71), the largest cluster, was defined as the mucocutaneous group, C2 (n = 20) as the arthritis group, C3 (n = 39) as the gastrointestinal group, in which all patients presented with intestinal lesions, and five cases with esophageal ulcers. In C4 (n = 22), showing a mixture of uveitis and vascular lesions, 15 patients presented with uveitis and 8 had vascular lesions, and 1 case had central nervous system lesions.Four phenotype clusters were identified. Patients with skin lesions comprised the largest cluster, while gastrointestinal, panuveitis, and cardiovascular clusters are the most commonly involved organs in late-onset BS patients. Key Points • Our analysis demonstrated the phenotype discrepancy between early and late onset groups. • Four phenotype clusters were identified, with gastrointestinal, panuveitis and cardiovascular clusters representing commonly involved organs.

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 P = 0.004), cardiac lesion (RR 5.46, 95% CI 2.33, 12.77, P P = 0.007) and was negatively associated with genital ulcers (RR 0.84, 95% CI 0.79, 0.91, P 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.

Objective The aim of this study is to examine the clinical features of patients with Behçet’s disease (BD) in the presence or absence of latent tuberculosis infection (LTBI). Methods This was a retrospective study of 232 consecutive patients with active BD hospitalized between October 2012 and June 2017. LTBI was diagnosed based on the positive T-SPOT.TB assay, negative clinical, and imaging examinations. Results Among the 232 patients, 68 (29.3%) had LTBI. The frequency, number, and scope of oral ulcers in the BD-LTBI group were significantly more serious than in the non-LTBI group (all P < 0.05). Genital ulcers and eye involvement in the LTBI group were significantly higher than in the non-LTBI group (both P < 0.01). No active TB was diagnosed during follow-up (median, 27.9 months; range, 3–58 months). The patients with LTBI had signs of liver damage compared with the non-LTBI group. In the LTBI group, the frequency of alanine transaminase >2.0, the upper limit of normal, was higher in the rifampicin subgroup compared with the non-rifampicin subgroup (P = 0.033). Conclusion Patients with BD and LTBI had worse clinical features than those with BD without LTBI. Rifampicin might be associated with the damage to liver in BD patients combined with latent TB.

To investigate the clinical characteristics and laboratory data in Behcet’s syndrome (BS) patients in China and analyze the risk factors of disease activity. A retrospective analysis method was used and the demographic data and laboratory results were collected from 174 BS patients. Univariate and multivariate logistic regression analyses were used to analyze the demographic data and laboratory indexes whether that are risk factors or not of disease activity. The most common clinical manifestations of BS patients enrolled were mouth ulceration (48.85%), followed by erythema nodosum (20.69%), and eye involvement (13.75%), while the least common was headache (0%). Most active BS patients (96.55%) used 2 or ≥ 3 immunosuppressants to control disease, while most inactive patients (75%) used 0 or 1 immunosuppressant. The associated risk factors of disease activity consisted of disease duration, neutrophil-to-lymphocyte ratio (NLR), white blood cells, red blood cells, hemoglobin, platelets, fibrin degradation products, IgG, IgM, complement 3, complement 4, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), albumin, albumin-to-globulin ratio (AGR), and high-density lipoprotein (HDL) (P < 0.05 or P = 0.00). Disease duration (≤ 60 months) (OR 8.49, 95% CI 2.09–34.49, P = 0.003), NLR (≥ 2) (OR 8.68, 95% CI 2.12–35.49, P = 0.003), CRP (≥ 10 mg/L) (OR 41.12, 95% CI 8.43–200.70, P = 0.000), ESR (≥ 20 mm/H) (OR 9.60, 95% CI 2.41–38.18, P = 0.001), and AGR (< 1.5) (OR 12.42, 95% CI 2.92–52.80, P = 0.001) were the independent risk factors of disease activity in BS patients. Attention should be paid to the risk factors of disease activity and the medicine should be adjusted correspondingly.

Summary: Background: Vascular Behçet's disease (VBD) might involve all sizes of arterial and venous vessels. Major vascular involvement caused the primary death in Behçet's syndrome (BS). We aimed to investigate the clinical characteristics and factors influencing the prognosis of VBD. Patients and methods: A retrospective analysis of the prospectively collected data of the Shanghai BS database from October 2012 to October 2018 was conducted. Patients who were diagnosed with BS and merged with venous thrombosis, arterial aneurysms, and arterial stenosis/occlusions were enrolled. Results: There were 47 patients with vascular involvement among 836 BS patients, 38 males and 9 females. The numbers of patients with venous thrombosis, arterial aneurysm, and arterial stenosis/occlusion were 25 (53.2 %), 21 (44.7 %), and 12 (25.5 %), respectively. Nearly half of the venous thromboses were located in limbs (n = 22, 46.8 %). Arterial aneurysm was the main form of arterial lesion. Most of the patients (93.6 %) were treated with corticosteroids and immunosuppressants. Late onset of BS or with arterial involvement had lower treatment response. Therapy with biological agents had significantly better results than that in the group without biological treatment (94.1 % vs. 80 %, P = 0.005). Conclusions: VBD showed a male preponderance and more than half of the patients presented with venous thrombosis. Late onset and arterial involvement were associated with poor prognosis. Therapy with biological agents is a viable alternative treatment to improve the prognosis.

Background Cardiac Behçet’s syndrome (cardiac BS) is rare but lethal. And the influencing factors of prognoses in cardiac BS are not clear. This study was conducted to summarize the features of cardiac BS and find the predictors of unfavourable prognoses for cardiac BS patients. Methods Sixty-six cardiac BS patients were included from 1467 BS patients in the Shanghai Behçet’s syndrome database. The median follow-up duration was 4.0 (2.0–7.0) years. The unfavourable prognosis was defined as a compound event containing all-cause death and rehospitalization related to worsened cardiac lesions or postoperative complications. Logistic regression analyses were performed to evaluate the predictors of unfavourable prognoses. Results In this study, the mortality rate of cardiac BS patients was 10.61% (7/66), and 22 (33.33%) patients experienced two or more surgeries. Ninety-six cases were collected from 66 cardiac BS patients. The effective rates of postoperative biological agents and Janus kinases (JAK) inhibitors in cardiac BS patients were 88.89% (16/18) and 81.25% (13/16) respectively. Former smoking, valve replacement or repairment and poor compliance were found to be the independent risk factors of unfavourable prognoses; preoperative immunosuppressive therapies and postoperative immunosuppressive therapies were independent protective factors for unfavourable prognoses in total cardiac BS. In 65 cases of cardiac BS after aortic valve surgeries, former smoking was an independent risk factor for unfavourable prognoses. Bentall procedure, postoperative traditional DMARDs, postoperative JAK inhibitors or biological agents were independent protective factors for unfavourable prognoses in cardiac BS patients after aortic valve surgeries. Conclusion Both preoperative immunosuppressive therapies and postoperative immunosuppressive therapies are necessary for cardiac BS patients. Bentall procedure is recommended to be performed in BS patients with severe AR. Besides biological agents, JAK inhibitors can be used in cardiac BS patients after aortic valve surgeries for promoting favourable outcomes.

BackgroundT lymphocytes are one of the major components of the adaptive immunity in Behçet’s syndrome (BS) pathology. To further understand of the role of T cells in Behçet’s syndrome (BS), we explore disease related T cell receptor (TCR) repertoires.MethodsWe performed a bulk sequencing of the TCR beta chain (TRB) of peripheral T-cells collected from 45 BS patients with panuveitis, intestinal involvement, and cardiovascular lesions and 10 cases of healthy controls (HC). Data analysis included peptide sequences, diversity analysis, variable (V)-joining (J) gene usage and K-nearest neighbor algorithm.ResultsWe found significant differences in V, J and V-J combinations between the BS patients and HC. The decrease in TCR clone diversity indicates clonal expansion in BS. Although no significant difference in TCR clone diversity was observed among patient subgroups, the patients with panuveitis displayed the highest heterogeneous TCR distribution. In addition, a set of V-J genes could effectively discriminate between active and inactive BS patients with an area under the receiver operating characteristics (ROC) curve of 0.88 (95% CI: 0.77-1.00).ConclusionsClonal T cell expansion has been observed in BS patients. TCR profiles could help in the discrimination between active and inactive BS.

To investigate the incidence, characteristics, and potential risk factors of anemia in patients with newly-diagnosed intestinal Behcet's disease (BD).In this cross-sectional study, 106 newly-diagnosed intestinal BD patients were identified, and a gender-, age- and organ involvement-matched control group of 241 non-intestinal BD patients was established. Hemoglobin (Hb) levels below 120 g/L in women and 130 g/L in men were diagnosed as anemia; these were further classified as mild (Hb ≥ 90 g/L), moderate (60 g/L ≤ Hb 90 g/L), and severe (Hb 60 g/L) anemia for both genders. The prevalence, type and severity of anemia in these patients were assessed. Logistic regression was performed to analyze the relationship between clinical variables and anemia in newly-diagnosed intestinal BD patients.The prevalence of anemia was 60.38% in newly-diagnosed patients with intestinal BD, significantly higher than those with non-intestinal BD (27.80%). Patients with intestinal BD had lower Hb, higher levels of C-reactive protein (CRP) and higher erythrocyte sedimentation rates (ESR) than did patients with non-intestinal BD (P 0.05). The majority of patients had mild-to-moderate anemia. The most common type of anemia found in both groups was normocytic normochromic anemia (56.25% for intestinal BD and 59.70% for non-intestinal BD). Multivariate logistic regression showed that the independent risk factors for anemia were disease activity index (DAIBD) (OR = 4.949, 95% CI: 1.504-16.282), higher levels of ESR (OR = 1.058, 95% CI: 1.019-1.099), and lower body mass index (BMI) (OR = 0.843, 95% CI: 0.727-0.977) for newly-diagnosed intestinal BD patients.Anemia is common in patients with newly-diagnosed intestinal BD. Although typically mild or moderate, anemia may closely relate with disease activity.

Background Takayasu arteritis (TAK) is a rare large vessel vasculitis, and epidemiological data on TAK are lacking in China. Thus, we designed this study to estimate the TAK prevalence and incidence in residential Shanghai, China. Methods Data on diagnosed TAK cases aged over 16 years were retrieved from 22 tertiary hospitals in Shanghai through hospital electronic medical record systems between January 1, 2015 and December 31, 2017 to estimate the prevalence and incidence. A systematic literature review based on searches in PubMed, Ovid-Medline, Excerpta Medica Database (EMBASE), Web of Science, and China National Knowledge Infrastructure (CNKI) was performed to summarize TAK distribution across the world. Results In total 102 TAK patients, with 64% female, were identified. The point prevalence (2015-2017) was 7.01 (95% CI 5.65-8.37) cases per million, and the mean annual incidence was 2.33 (1.97-3.21) cases per million. The average age of TAK patients was 44 ± 16 years, with the highest prevalence (11.59 [9.23-19.50] cases per million) and incidence (3.55 [0.72 3.74] cases per million) in the 16 to 34 years population. Seventeen reports were included in the system review, showing that the epidemiology of TAK varied greatly across the world. The incidence and prevalence were both relatively higher in Asian countries, with the prevalence ranging 3.3-40 cases per million and annual incidence ranging 0.34-2.4 cases per million. Conclusions The prevalence and incidence of TAK in Shanghai was at moderate to high levels among the previous reports. The disease burden varied globally among racial populations.

MicroRNAs (miRNAs) derived from plasma exosomes are potential diagnostic biomarkers. However, little is known about the expression of miRNAs derived from plasma exosomes in patients with intestinal Behçet's syndrome (BS). This study aimed to explore the difference of miRNAs derived from plasma exosomes between intestinal BS patients and healthy people, and further identify potential biomarkers that predict the disease activity of intestinal BS.A total of 43 intestinal BS patients and 23 healthy volunteers were enrolled, among whom 23 were active intestinal BS and 20 were stable intestinal BS. The miRNAs expression profiles of plasma exosomes in 3 active intestinal BS patients and 3 healthy volunteers were determined using next-generation high throughput sequencing. Additionally, significantly differentially expressed miRNAs were further analysed by quantitative real-time polymerase chain reaction (qRT-PCR) in a validation cohort of 60 subjects.From the sequencing analysis, 15 miRNAs were identified to be differently expressed (p0.05). Of these, 13 miRNAs were up-regulated, and 2 were down-regulated in intestinal BS patients compared with healthy volunteers. Furthermore, qRT-PCR analysis confirmed that miR-141-3p was down-regulated and miR-122-5p, miR-150-3p, miR-183-5p, miR-224-5p and miR-342-5p were up-regulated in intestinal BS patients' plasma exosomes. Additionally, the level of miR-141-3p was negatively correlated with disease activity indicators of intestinal BS, while miR-122-5p, miR-150-3p, miR-183-5p, miR-224-5p and miR-342-5p was positively correlated with disease activity indicators of intestinal BS.Circulating miR-141-3p, miR-122-5p, miR-150-3p, miR-183-5p, miR-224-5p and miR-342-5p derived from plasma exosomes may serve as biomarkers of disease activity in intestinal BS.

Intestinal Behçet's syndrome (IBS) has high morbidity and mortality rates with serious complications. The purpose of this study was to investigate the expression of pyroptosis-related proteins in the intestinal tissues of IBS patients and explore the role of plasma exosomes derived from IBS patients in the pyroptosis of intestinal epithelial cells.Immunohistochemistry was used to investigate the expression of nucleotide-binding domain-like receptor protein 3 (NLRP3), caspase-1, and gasdermin D (GSDMD). Quantitative real-time PCR was employed to measure the mRNA levels of IL-1β and IL-18 in the intestinal tissues. Plasma exosomes were isolated and observed by transmission electron microscopy. The exosomes were co-cultured with intestinal epithelial cells in vitro. Western blot was used to measure the expression of pyroptosis-related proteins including NLRP3, full-length GSDMD, N-terminal GSDMD, pro-caspase-1, and cleaved caspase-1. The levels of IL-1β and IL-18 were detected by enzyme-linked immunosorbent assay. Cell death was measured by using the lactate dehydrogenase (LDH) release assay.Expression of NLRP3 (12.2% ± 1.2%, 8.1% ± 0.9%, t = 4.692, p = 0.009), caspase-1 (24.6% ± 2.1%, 4.2% ± 1.8%, t = 12.842, p = 0.000), and GSDMD (16.6% ± 1.9%, 9.8% ± 1.3%, t = 5.194, p = 0.007) were significantly increased in the intestinal tissues of patients with IBS compared with normal control (NC) group, respectively. The relative mRNA levels of IL-1β (t = 4.308, p = 0.005) and IL-18 (t = 3.096, p = 0.021) in the intestinal tissues were significantly higher in IBS patients than in NC group, while the protein levels of IL-1β (t = 3.873, p = 0.018) and IL-18 (t = 4.389, p = 0.012) were also significantly increased, which was consistent with the results of the relative mRNA levels. Moreover, we found that exosomes from IBS patients significantly induced pyroptosis of intestinal epithelial cells via the activation of NLRP3 inflammasome in vitro experiments.Plasma exosomes derived from IBS patients may induce pyroptosis of intestinal epithelial cells via the activation of NLRP3 inflammasome. Key Points •The role of exosomes in IBS is first reported in this study. • In this study, we explored the mechanism that plasma exosomes derived from IBS patients may induce pyroptosis of intestinal epithelial cells via the activation of NLRP3 inflammasome.

Objectives: Behçet syndrome (BS) is heterogeneous with clinical variability across ethnicities and geographic locations. This study analyzed the clinical characteristics of BS associated with cytopenia and trisomy 8.Methods: This was a retrospective study of Chinese BS patients in the Shanghai Behçet’s disease database who were diagnosed with cytopenia and trisomy 8 between October 2012 and April 2019. All subjects fulfilled the International Study Group (ISG) criteria or the International Criteria for Behçet’s Disease (ICBD).Results: Twenty-one patients with BS had cytopenia with trisomy 8; 16 (16/21, 76.2%) were diagnosed with myelodysplastic syndrome (MDS). Compared with 1,147 patients with BS without cytopenia, patients with BS and trisomy 8/cytopenia were more likely to be female (23.8% vs 51.8%,p= 0.011), older (37.9 ± 12.8 vs 45.1 ± 12.0, p = 0.007), have fewer skin lesions (33.3% vs 56.7%,p= 0.033), fever (52.4% vs 1.1%, p < 0.001), have higher C-reactive protein, have higher erythrocyte sedimentation rate (ESR), and display a higher rate of gastrointestinal (GI) ulceration (61.9% vs 9.2%, p < 0.001). By contrast, they had a lower leukocyte count, lower hemoglobin levels, and a lower platelet count (p < 0.05 for all comparisons). Ulcers in the ileocecal region were more frequently seen in intestinal patients with BS with trisomy 8 than in patients with BS without cytopenia (100.0% vs 41.8%; p = 0.002). Compared with 54 patients with MDS without BS symptoms, those with BS and trisomy 8-cytopenia were more likely to be female (23.8% vs 70.4%, p< 0.001) and younger (37.9 ± 12.8 vs 67.2 ± 12.4, p= 0.001). In the follow-up, three patients with BS with trisomy 8 (3/21, 14.3%) died, which is a higher rate than in patients with BS (5/1147, 0.44%) and much lower than in patients with MDS (13/54, 24.1%).Conclusions: The data on patients with BS with cytopenia and trisomy 8 were different from BS or MDS in epidemiology, clinical performance, and prognosis. BS with cytopenia and acquired trisomy 8 may be a new clinical syndrome.

We carefully read the recent study by Liu et al on the effectiveness of tofacitinib for refractory Behcet’s syndrome (BS).1 In total, 13 patients with BS were enrolled, including 5 cases with vascular involvement, 7 with gastrointestinal (GI) ulcers and one suffering polyarthritis. As an add-on approach, tofacitinib improved the outcomes in all patients with vascular lesions, but unchanged or worsened in 5/7 GI involvement cases. Their observation give support to the hypothesis that BS is not an independent disease, but a heterogeneous and multisystemic complex syndrome.2 Different major organ involvements, vascular and GI involvement could link to its unique underlying immunopathological mechanisms, which led to radical treatment responses.3 In their study, the only manifestation of ocular involvement was scleritis in one patient with polyarthritis, which is not a specific ophthalmological feature of BS. Notably, uveitis is one of the specific features of ocular involvement in patients with BS,4 which may lead to visual loss in up to 25% of patients in spite of biological agents.5 Here, we reported the efficacy and safety profile of …

Background: Intestinal Behçet’s syndrome (BS) has high morbidity and mortality rates with serious complications. This study aimed to investigate the clinical characteristics and laboratory indicators of intestinal BS compared with mucocutaneous BS patients in China and analyzed the risk factors of intestinal complications in BS patients.Methods: A retrospective analysis was used to collect the demographic data and laboratory results from 97 patients newly diagnosed with intestinal BS and 154 patients newly diagnosed with mucocutaneous BS. Univariate and multivariate logistic regression analyses were used to analyze the demographic data and laboratory indexes whether a risk factor of intestinal involvement in BS patients.Results: The most common clinical manifestations of first onset in intestinal BS patients were oral ulceration (100.00%), followed by genital ulcers (62.89%) and erythema nodule (28.87%), gastrointestinal lesions (28.87%), pseudofolliculitis (25.77%), fever (17.53%), arthritis (16.49%), ocular involvement (5.15%), while the least common were vascular involvement (2.06%) and blood system involvement (2.06%). The most common intestinal segment involved in intestinal BS patients was terminal ileum (30.9%), followed by ileocecal (18.6%), colon (15.5%). Most intestinal BS patients (89.7%) used ≥ 3 immunosuppressants to control disease while most mucocutaneous BS patients (92.9%) used 1 or 2 immunosuppressant. By univariate logistic regression analysis, we found gender, age at hospitalization, age of disease onset, BDCAF, T-SPOT, fever, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), white blood cell (WBC), red blood cells (RBC), hemoglobin (HGB), neutrophil-to-lymphocyte ratio (NLR), serum amyloid A (SAA), complement 3 (C3), albumin, total cholesterol(TCH), high-density lipoprotein(HDL) and interleukin 6 (IL-6) were all risk factors of intestinal involvement in BS patients (P10mg/L), HGB (7pg/ml) were the independent risk factors of intestinal involvement in BS patients (all PConclusions: More attention should be paid to gender, BDCAF, ESR, CRP, HGB and IL-6 of intestinal involvement in BS patients and therapeutic regiment should be adjusted timely to prevent the occurrence of serious clinical complication in BS patients.

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.

This study aimed to investigate the predictive factors for uveitis recurrence (UR) risk in Behcet's disease (BD) patients. BD patients (n=164) with a history of uveitis were recruited, and demographic data, clinical features, and laboratory tests were recorded. Uveitis was defined as anterior uveitis, intermediate uveitis, posterior uveitis, panuveitis referring to the "International Uveitis Study Group recommendations for the evaluation of intraocular inflammatory disease". In total, there were 70 UR patients and 94 non-UR patients. Compared to non-UR patients, UR patients appeared to be older and presented with increased uveitis occurrence rate and times within 3 months, oral ulcers occurrence rate, as well as higher concentrations of triglycerides (TG), total cholesterol (TC), low-density lipoprotein (LDL), and serum amyloid A (SAA). Multivariate logistic model disclosed that uveitis occurrence times within 3 months, oral ulcers, TG, LDL, and SAA independently predicted higher risk of UR. Furthermore, receiver operating characteristic curve analysis showed that the combination of uveitis occurrence times within 3 months, oral ulcers, TG, LDL, and SAA exhibited a high predictive value for UR risk with an area under the curve of 0.983 (95%CI: 0.969-0.998). In conclusion, uveitis occurrence times within 3 months, oral ulcers, TG, LDL, and SAA might be potential predictive factors for UR risk in BD patients, which can help in prevention and management of the disease.

Background The Clinical features of vascular Behcet’s disease (BD) are not well understood because there are few studies. Our study aimed to investigate characteristics of vascular BD in both genders in different age groups. Results We enrolled 923 patients with BD who presented to our hospital with adequate medical histories and proper vascular screening exams. The raw incidence rate of vascular BD was 17.98% (166/923). The ratio of vascular BD in male to female patients was 1.868 (p = 0.0004, 95% confidence interval (CI): 1.317 to 2.625). There was a tendency towards higher ESR and CRP in vascular BD patients than in mucocutaneous, but the difference was not significant. The most susceptible affected vessels were cerebral (29.6% in males, 59.4% in females) and lower limb vessels (31.2% in males, and 17.2% in females). The incidence of vascular involvement in younger (

Background Patients undergoing anti-tumor necrosis factor (TNF) treatment are more susceptible to latent tuberculosis infection (LTBI). The aim of the current study was to determine the rate of active tuberculosis (TB) in patients with Behcet's disease (BD) pre- and post-anti-TNF treatment and to evaluate the long-term efficacy of LTBI screening as primary prophylaxis in China. Methods This retrospective study included BD patients eligible for anti-TNF therapy at a single institution in Fudan University, China. On the basis of the results of T-SPOT.TB assay, chest radiograph, and history of exposure to TB, patients were screened and regularly followed up at 3-months interval. Results Eighty-nine BD patients with mean disease duration of 87.5 ± 86.1 months were included. Their median duration of anti-TNF therapy was 10.6 months; 51 patients were treated with Infliximab, 38 with Etanercept, and four with Adalimumab. While 84 patients received a consecutive single anti-TNF drug therapy, five patients switched to a second drug. Twelve patients demonstrated positive results in LTBI screening: three had history of TB exposure and nine were solely T-SPOT.TB-positive patients. Before anti-TNF treatment, LTBI treatment was initiated in 11 patients, and one patient refused treatment. With a median follow-up period of 27.9 months, we observed only one case (1.1%) of intestinal TB during Infliximab treatment. Conclusion Regardless of anti-TNF treatment, long-term screening via T-SPOT.TB assay might represent a more sensitive approach to identify BD patients with LTBI. As a secondary prophylaxis, the LTBI treatment is effective in a country with high risk of TB.

Background/aim Differentiating intestinal Behcet’s disease (BD) from Crohn’s disease (CD) is highly challenging, as they often mimic each other in terms of clinical manifestations. Endoscopy is an important modality for distinguishing bowel lesions. The study was designed to identify clinical manifestations that are easily confused and to evaluate the efficacy of endoscopy for distinguishing intestinal BD from CD by several overlapping signs. Materials and methods The data from 111 patients with intestinal BD and 81 patients with CD were retrospectively analyzed. Logistic regression was applied to establish a prediction model based on endoscopic findings for the differential diagnosis. The diagnostic efficacy of endoscopy was verified using the area under the receiver operating characteristic (ROC) curve. Results Among intestinal BD patients mucocutaneous lesions were the leading clinical manifestations. Gastrointestinal symptoms were common in CD but were rare in intestinal BD (P < 0.001). CD patients with moderate-to-severe activity were more common than intestinal BD patients presenting with equivalent activity (P < 0.05). Independent factors that distinguished intestinal BD from CD were solitary ulcer in the ileocecal area (P < 0.001), perianal abscess (P = 0.049), single segment (P < 0.001), round intestinal ulcer (P = 0.013), intestinal obstruction (P = 0.035), and fistula (P < 0.001). The scores ranged from –2 to 3. The area under the ROC curve was 0.874 (95% CI: 0.823–0.926) (P < 0.001). With a score of 1.5 as the diagnostic cutoff value, the sensitivity and specificity were 76.3% and 80.6%, respectively. Conclusion Mucosal injuries were rarer in patients with intestinal BD than in those with CD. The differentiation model combining several endoscopy features appeared to be reliable for distinguishing between intestinal BD and CD.

OBJECTIVE: This study aimed to investigate the correlation of serum uric acid (SUA) with risk and dilatation diameter of ascending aortic dilatation (AAD) in Behçet's disease (BD) patients. METHODS: Seventeen BD patients complicated with AAD and 20 BD patients without AAD were consecutively enrolled and categorized into AAD group and control group, respectively. Ascending aortic dilatation was determined by two‐dimensional doppler echocardiographic examination, and AAD was defined as a diameter of ascending aorta ≥3.8 and

To compare the efficacy and the safety of low-dose (3.5 mg/kg) or standard-dose (5 mg/kg) of infliximab (IFX) for induction and maintenance treatment in Chinese patients with intestinal Behcet's disease (BD), and to identify potential predictors to corticosteroid-free clinical remission at week 30.A prospective trial was conducted in 20 patients with moderate-to-severe active intestinal BD, followed up for 30 weeks. Ileocolonoscopic examinations were done at baseline and week 14. A logistic regression model was used to assess the predictors of corticosteroid-free remission at week 30.As the primary end point, steroid-free remission at week 30 was observed in 40% and 60% of patients who received low and standard doses of IFX, respectively (Low-dose of IFX is effective and safe for induction and maintenance therapy in patients with active intestinal BD. MH at week 14 predicted clinical remission at week 30.

The automatic control system is one of the most important parts of improve cold rolling strip precision and increase the automate level. The improved cold strip mill process control system has been developed and successfully applied to several new cold strip production lines. This paper introduces the Key Technical Features and New Technology of the system include the system architecture, hardware configuration and new control algorithms, etc. System operation and actual production proves that the system has the features of shorter commissioning time, convenient maintenance, high reliability, high automatic level and control precision.

Rationale:Intestinal Behcet's disease (BD) is characterized by intestinal ulcerations and gastrointestinal symptoms. Ulcerative intestinal tuberculosis (TB) is usually with dyspepsia, abdominal pain, vomiting, and weight loss. The 2 diseases exhibit similar clinical manifestations, but the m

Polymorphisms in the Interleukin (IL)-1-related genes at the locations -889, -511, + 3962 and mspa1l 1100 have been investigated for possible association with Behçet's disease (BD).A literature-based search was conducted to identify all relevant studies. Five independent studies from Turkish population met the included criteria.IL-1α -889 CT [odds ratio (OR) = 0.72, 95% confidence interval (CI) = 0.55-0.95], IL-1α -889 TT (OR = 0.61, 95% CI = 0.40-0.93), IL-1β + 3962 C (OR = 1.41, 95% CI = 1.07-1.88), IL-1β + 3962 T (OR = 0.71, 95% CI = 0.53-0.94) IL-1β + 3962 CC (OR = 2.08, 95% CI = 1.08-3.99), IL-1β + 3962 CT (OR = 0.58, 95% CI = 0.38-0.88), IL-1 receptor antagonist (IL-1 Ra) mspa1l 1100 CT (OR = 0.69, 95% CI = 0.49-0.96), IL-1 Ra mspa1l 1100 TT (OR = 1.50, 95% CI = 1.08-2.08) had a significant association with BD. The pooled estimates for IL-1α -889 C, IL-1α -889 CC, IL-1α -889 T had a non-significant association with BD.IL-1α -889 CT, IL-1α -889 TT, IL-1β + 3962 C, IL-1β + 3962 T, IL-1β + 3962 CC, IL-1β + 3962 CT, IL-1 Ra mspa1l 1100 CT, IL-1Ra mspa1l 1100 TT promoter polymorphisms may confer susceptibility to BD in Turkish population.

Behcet's disease is a condition characterized by systemic vasculitis liable to thrombosis, and may involve the parenchyma and blood vessels in the brain. We report a case of Behcet's disease that progressed into central neural system involvement rapidly due to failure of tertiary prevention. This case demonstrates the importance of management of the high risk factors of thrombosis and timely prevention of disease progression.

To examine the expression of miRNAs and mRNAs of heme oxygenase 1 (HO-1) in patients with active intestinal Behcet's disease (BD).Peripheral blood mononuclear cells (PBMCs) were obtained from BD patients with active ileocecal ulcers or from healthy donors. Expression levels of four miRNAs were studied using real-time PCR. The levels of mRNAs of ho-1, bach1, and CD14 were measured by qRT-PCR. Serum levels of cytokines were analyzed by ELISA.Among four miRNAs, only levels of miR-196a2 were significantly decreased from BD patients with active ileocecal ulcers as compared with healthy controls. Moreover, level of mRNA ho-1 expression in PBMCs from patients with BD was reduced. No significant difference on bach1 and CD14 mRNA levels was observed. Levels of IFN-γ, IL-17, IL-10, IL-1β, and TNF-α were higher in patients with active intestinal BD than those in healthy controls.The present results suggest that miR-196a2 expression is decreased in active intestinal BD patients. Down regulated miR-196a2 may be involved in intestinal BD pathogenesis by targeting Bach1/ho-1. Consequently, pro-inflammatory cytokines are closely implicated in the evolution of intestinal BD.

Intestinal Behcet’s disease (BD) is a specific subtype of BD. Effective drug therapy for intestinal BD remains elusive. To investigate long-term outcomes and identify predictors of sustained response in intestinal BD patients receiving infliximab (IFX) treatment. The medical records were reviewed of patients received IFX from September 2012 to March 2016. The cumulative probabilities of sustained response were calculated using the Kaplan–Meier. Predictor factors for sustained response were accessed by receiver operating characteristic curve. Totally, 27 active intestinal BD patients were enrolled. Sustained responses were observed in 17 patients, after a median follow-up duration 24 months (interquartile range 9–37). The proportion of clinical remission at week 14, 30, and 52 had occurred in 84.6, 70, and 70%, respectively, with the proportion of clinical remission of 69.2, 40, and 55%. The mucosal healing (MH) rate at week 14 was 72%. Kaplan–Meier estimated patients with achievement of clinical and biological responses at week 14 or MH was likely to remain sustained clinical response. ROC curve analysis revealed CRP level (of 6.85 mg/L) at week 14 is a potential predictor for discriminating patients with sustained response from relapse, with an area under the curve values of 0.837. IFX is effective and safe for induction and maintenance therapy in Chinese patients with moderate-to-severe active intestinal BD. Early achievement of clinical response and mucosal healing might associate long-term response. A lower CRP level seems to be associated with a more benign clinical course.

The new fuzzy PID control system presented in this thesis is a combination of the models used in traditional temperature control system and Dual cross limiting temperature control system that took problems such as the slow response and overshoot of furnace temperature caused by the drastic change of the value of gas and the pressure in to account. The fuzzy PID control system helps to conquer the drawbacks of Poor dynamic tracking performance and has the distinction of being flexible, quick and adaptable. It is also proven by practice that, it is environmental friendly and energy-saving with the high-accuracy and lower consumption of this new control system.

Objective. To analyse the potential risk factors of nosocomial infections in patients with active rheumatoid arthritis (RA). Methods. A total of 2452 active RA patients at Hospitals in Shanghai between January 2009 and February 2011 were analyzed. Their demographic and clinical characteristics were compared with those without infection, and the potential risk factors were determined by logistic regression analysis. Results. Multivariate analysis indicated the gender (OR=0.70, 95% CI 0.53–0.92), duration in hospital (OR=1.03, 95%CI 1.01–1.05), number of organs involved (OR=0.82, 95%CI 0.72–0.92), number of disease-modifying antirheumatic drugs ((DMARDs) (OR=1.22, 95%CI 1.061–1.40)), corticosteroid therapy (OR=1.02, 95%CI 1.01–1.03), peripheral white blood cell counts ((WBC) (OR=1.04, 95%CI 1.00–1.08)), levels of serum albumin (OR=0.98, 95%CI 0.97–0.99), and C-reactive protein ((CRP) (OR=1.03, 95%CI 1.01–1.04)) that were significantly associated with the risk of infections. Conclusion. The female patients, longer hospital stay, more organs involved, more DMARDs, corticosteroid usage, high counts of WBC, lower serum albumin, and higher serum CRP were independent risk factors of infections in active RA patients.