Yan Pengguang, Li Kemin, Cao Yang, Wu Dong, Li Ji, Qian Jiaming, Zhou Weixun, Li Jingnan
Key Laboratory of Gut Microbiota Translational Medicine Research, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Peking Union Medical College, Beijing, China.
Front Med (Lausanne). 2022 Aug 11;9:926800. doi: 10.3389/fmed.2022.926800. eCollection 2022.
There are few reports on standard treatment and long-term prognosis in patients with cryptogenic multifocal ulcerative stenosing enteritis (CMUSE), particularly in patients in whom remission could not be induced by steroids. The aim of this study was to evaluate the treatment response and progression-free periods of patients with CMUSE and to identify the factors predictive of steroid resistance.
This was a retrospective cohort study that included 25 patients with clinically confirmed CMUSE between 1984 and 2021 from the enteropathy clinic of a tertiary care center. For statistical analyses, chi-square test or Fisher's exact test were used for categorical variables. Survival curves were plotted using the Kaplan-Meier method.
The overall median progression-free period was 48 months (range, 1-108 months) after comprehensive therapy, and initial manifestation with severe bleeding rather than ileus was associated with the long-term efficacy. Patients with steroid resistance ( = 10, 55.6%) had poor prognosis, and non-responders had more favorable baseline clinical characteristics, with a higher percentage of female patients (60% vs. 12.5%), earlier disease onset (26.5 years vs. 39 years), rapid progression (42 vs. 108 months), severe anemia (80% vs. 50%), and hypoalbuminemia (50% vs. 0%), in accord with lymphangiectasia or angioectasia identified in pathology.
There is no guaranteed treatment strategy in the maintenance of long-term clinical remission for CMUSE patients, particularly in whom with steroid resistance. Female patients with early symptoms onset, severe gastrointestinal hemorrhage and hypoalbuminemia seem to have poor long-term prognosis.
关于隐源性多灶性溃疡性狭窄性肠炎（CMUSE）患者的标准治疗和长期预后的报道较少，尤其是那些无法通过类固醇诱导缓解的患者。本研究的目的是评估CMUSE患者的治疗反应和无进展生存期，并确定预测类固醇抵抗的因素。
这是一项回顾性队列研究，纳入了1984年至2021年间在一家三级医疗中心的肠病门诊临床确诊的25例CMUSE患者。对于统计分析，分类变量采用卡方检验或Fisher精确检验。生存曲线采用Kaplan-Meier方法绘制。
综合治疗后总体中位无进展生存期为48个月（范围1 - 108个月），以严重出血而非肠梗阻为初始表现与长期疗效相关。类固醇抵抗患者（n = 10，55.6%）预后较差，无反应者基线临床特征更有利，女性患者比例更高（60%对12.5%）、疾病发病更早（26.5岁对39岁）、进展更快（42个月对108个月）、严重贫血（80%对50%）和低白蛋白血症（50%对0%），与病理检查发现的淋巴管扩张或血管扩张一致。
对于CMUSE患者，尤其是类固醇抵抗患者，在维持长期临床缓解方面没有保证的治疗策略。症状出现早、严重胃肠道出血和低白蛋白血症的女性患者似乎长期预后较差。
