Department of RHEUMATOLOGY and Immunology, Huadong Hospital affiliated to Fudan University, #221 yan'an west Road, Shanghai, 200040, People's Republic of China.
Orphanet J Rare Dis. 2019 Nov 28;14(1):274. doi: 10.1186/s13023-019-1228-9.
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.
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.
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]).
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.
白塞病（ABD）是一种免疫性复发性系统性血管炎，具有慢性病程。我们研究了中国非手术肠道 ABD 患者长期复发、不良结局和无事件生存的预测因素。
这是一项前瞻性队列研究，纳入了 2012 年 10 月至 2019 年 1 月期间在我院就诊的 109 例符合 ABD 国际标准且结肠镜检查证实有肠道溃疡的肠道 ABD 患者。使用逻辑回归模型和 Cox 比例风险回归模型分别计算复发和不良结局、无事件生存的预测因素。
66 例肠道 ABD 患者（60.55%）有回盲部溃疡；19 例（17.43%）表现为结直肠溃疡；24 例（22.02%）同时有回盲部和结直肠溃疡。7 例（6.42%）至少有 1 次肠道溃疡复发。38 例（34.86%）有肠道溃疡不愈合。多因素分析显示，肠道溃疡位置（回盲部和结直肠）（比值比（OR）7.498[95%置信区间（95%CI）1.844-30.480]）、红细胞沉降率（ESR）>24mm/h（OR 5.966[95%CI 1.734-20.528]）、使用英夫利昔单抗（IFX）（OR 0.130[95%CI 0.024-0.715]）和治疗依从性差（OR 11.730[95%CI 2.341-58.781]）与不良结局独立相关。中位随访 28 个月后，45 例肠道 ABD 患者（41.28%）发生不良事件。与无事件生存时间较短相关的独立因素是 ABD 发病早（<7 岁）（风险比（HR）2.431[95%CI 1.240-4.764]）和治疗依从性差（HR 3.058[95%CI 1.612-5.800]）。
肠道溃疡（回盲部和结直肠）分布、ESR>24mm/h、不使用 IFX 治疗和治疗依从性差是肠道非手术 ABD 患者不良结局的独立危险因素。
