Clinical characteristics and factors influencing the prognosis of Behçet's disease complicated with vascular involvement.

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.

Poor prognostic factors in patients with newly diagnosed intestinal Adamantiades-Behçet's disease in the Shanghai Adamantiades-Behçet's disease database: a prospective cohort study.

BACKGROUND: Adamantiades-Behçet's Disease (ABD) is an immunological recurrent systemic vasculitis with a chronic course. We investigated the predictors of long-term flare-ups, poor outcomes and event-free survival in Chinese non-surgical patients with intestinal ABD. METHODS: This was a prospective cohort study of 109 intestinal ABD patients seen in our institution between October 2012 and January 2019 who met the international criteria for ABD and had intestinal ulcers confirmed on colonoscopy. Predictors of relapses and poor outcomes, event-free survival were calculated using logistic regression models and Cox proportional hazard regression models, respectively. RESULTS: Sixty-six intestinal ABD patients (60.55%) had ileocecal ulcers; 19 patients (17.43%) presented with colorectum ulcers; 24 patients (22.02%) showed both ileocecal and colorectum ulcers. 7 patients (6.42%) experienced at least 1 flare-up of intestinal ulcers. 38 patients (34.86%) complained of non-healing intestinal ulcers. In multivariate analysis, location of intestinal ulcers (ileocecal and colorectum) (odd ratio (OR) 7.498 [95% confidence interval [95% CI] 1.844-30.480]), erythrocyte sedimentation rate (ESR) > 24 mm/h (OR 5.966 [95% CI 1.734-20.528]), treatment with infliximab (IFX) (OR 0.130 [95% CI 0.024-0.715]), and poor compliance (OR 11.730 [95% CI 2.341-58.781]) were independently correlated with a poor outcome. After a median follow-up of 28 months, 45 intestinal ABD patients (41.28%) underwent adverse events. Factors independently associated with shorter event-free survival were early onset of ABD (< 7 years) (hazard ratio (HR) 2.431 [95% CI 1.240-4.764]) and poor compliance (HR 3.058 [95% CI 1.612-5.800]). CONCLUSION: Distribution of intestinal ulcers (ileocecal and colorectum), ESR > 24 mm/h, treatment without IFX, and poor compliance were independent risk factors for poor outcomes in non-surgical intestinal ABD patients.

High expression of APC is an unfavorable prognostic biomarker in T4 gastric cancer patients.

BACKGROUND: Adenoma polyposis coli (APC) mutation is associated with tumorigenesis via the Wnt signaling pathway. AIM: To investigate the clinical features and mechanism of APC expression in gastric cancer (GC). METHODS: Based on APC expression profile, the related genome-wide mRNA expression, microRNA (miRNA) expression, and methylation profile in GC, the relationship between APC and GC, as well as the prognostic significance of APC were systematically analyzed by multi-dimensional methods. RESULTS: We found that high expression of APC (APC high) was significantly associated with adverse outcomes of T4 GC patients. Genome-wide gene expression analysis revealed that varying APC expression levels in GC were associated with some important oncogenes, and corresponding cellular functional pathways. Genome-wide miRNA expression analysis indicated that most of miRNAs associated with high APC expression were downregulated. The mRNA-miRNA regulatory network analysis revealed that down-regulated miRNAs affected their inhibitory effect on tumor genes. Genome-wide methylation profiles associated with APC expression showed that there was differential methylation between the APC high and APC low groups. The number of hypermethylation sites was larger than that of hypomethylation sites, and most of hypermethylation sites were enriched in CpG islands. CONCLUSION: Our research demonstrated that high APC expression is an unfavorable prognostic factor for T4 GC patients and may be used as a novel biomarker for pathogenesis research, diagnosis, and treatment of GC.

Demography of vascular Behcet's disease with different gender and age: an investigation with 166 Chinese patients.

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 (< 50 years old) and older (≥ 50 years old) patients were similar, with ratios of 16.58% (122/736) and 23.53% (44/187) respectively. However, in females, younger patients were less likely to have vascular involvement than were older patients (11.43% vs. 20% p = 0.0328, OR: 0.5161, 95% CI: 0.2874 to 0.912). Aneurysm or pseudoaneurysm was diagnosed in 1.84% (17/923) patients, mostly in male patients (p < 0.05, OR: 3.221, 95% CI: 1.097 to 9.112). Twenty vascular BD patients were followed up, and the age at BD diagnosis was 33.23 ± 11.56 year. This did not differ statistically with their age at vascular involvement (36.15 ± 9.52 years). Ages of vascular BD patients did not differ significantly from those of mucocutaneous BD patients (n = 143) in both males and females. CONCLUSION: Vascular BD, including lethal types of aneurysm is more likely to occur in male patients. The female patients has a similar incidence rate with the males in their postmenopausal age. There was no evidence of progression course from mucocutaneous BD to vascular involvement.

Effectiveness and safety of Glycyrrhizae Decoction for Purging Stomach-Fire in Behcet disease patients: Study protocol for a randomized controlled and double-blinding trail.

BACKGROUND: Behcet disease (BD) is a worldwide-occurred autoimmune disorder and currently lack of optional successful treatment. An ancient traditional Chinese medical formula called Glycyrrhizae Decoction for Purging Stomach-Fire (GDPSF) was recorded and nowadays has been observed to be effective for BD patients. However, the strict randomized controlled and double-blinding trail is needed to further assess this alternative medicine. METHODS: To ascertain the potential effects and safety of GDPSF for BD patients and to determine whether combination application of GDPSF and thalidomide could possibly reduce the side effects and increase effectiveness for BD management, we will conduct a randomized, double blind, controlled clinical trial. Patients enrolled will be randomly assigned into 3 groups: GDPSF group, thalidomide group, and integrative group (treated by both GDPSF and thalidomide). Participants will receive treatment for 6 months and accept a 12 months follow-up. Before and after treatment, clinical manifestations, blood tests, thalidomide dosage, remission levels, quality of life, and satisfactory levels will be assessed. The data of assessments on each group before and after treatments will be collected and analyzed through historical control, while between groups through intergroup control. Then statistical analysis will be applied to assess the effects and safety. DISCUSSION: This study protocol will assess the effects and safety of GDPSF for BD patients GDPSF. Combination application of GDPSF and thalidomide might be a new integrative medical method for BD patients. TRIAL REGISTRATION: Chinese Clinical Registry (ChiCTR-ONC-16009621) on Oct. 2016 http://www.chictr.org.cn/showproj.aspx?proj=16395.