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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.

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.

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.

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