No abstract

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 (P<0.05 or P=0.00). Additionally, gender (male), BDCAF (≥2), ESR (≥15mm/H), CRP (>10mg/L), HGB (<130g/L) and IL-6 (>7pg/ml) were the independent risk factors of intestinal involvement in BS patients (all P<0.05).Conclusions: 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: This study aimed to describe the characteristics, treatment, and outcome of hematological involvement and assess possible association to define a “cytopenia phenotype” in BS patients. Methods: This was a retrospective study of BS patients in Shanghai Behçet syndrome database who were diagnosed with hematological involvement between October 2012 and December 2021. Results: A total of 40 BS patients with cytopenia were included (2.1%, 40/1950), the median follow-up duration was 35 (3-92) months. They presented high rate of trisomy 8 in the bone marrow (n=33, 82.5%). BS patients with cytopenia were more likely to be older age, female and have fewer skin lesions. Gastrointestinal (GI) ulceration, fever and high level of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were more frequent in BS patients with cytopenia. Ulcers in the ileocecal, small intestine and colon region and symptoms such as abdominal pain, weight loss and fever were more frequently seen in intestinal BS patients with cytopenia. All these cytopenia BS patients received standard guideline treatment regimens. In the follow-up, eight BS patients with cytopenia (8/40, 20.0%) died, 9 (22.5%) had not improved. As compared to alive BS patients, died BS patients were more likely to be older age and have high level of ESR.Conclusions:BS patients with cytopenia displayed a high rate of somatic mutations trisomy 8, GI involvement and high risk of death. Management of cytopenia was highly heterogeneous and many of the current treatment options are highly variable and suboptimal.