溃疡性结肠炎合并原发性硬化性胆管炎的临床特点并文献复习

Ulcerative colitis with primary sclerosing cholangitis:six cases report and literature review

目的:总结溃疡性结肠炎(UC)合并原发性硬化性胆管炎(PSC)的临床特点。方法:回顾性分析浙江大学医学院附属邵逸夫医院2017年5月至2022年5月收治的6例UC合并PSC患者的临床特征及诊治过程,并以"原发性硬化性胆管炎"、"溃疡性结肠炎"、"ulcerative colitis"、"primary sclerosing colangitis"为关键词检索中国知网、万方数据、PubMed数据库截止至2022年8月的文献,进行相关文献复习及总结。结果:纳入6例UC合并PSC患者,男性4例,女性2例;4例UC先于PSC确诊,2例同时确诊。6例均为广泛结肠型(E3),2例患者直肠豁免,4例患者有倒灌性回肠炎表现。5例患者碱性磷酸酶(AKP)升高。所有患者给予熊去氧胆酸(UDCA)口服治疗。2例患者合并胆管癌,随访1年内去世。共检索到75篇相关文献。结论:UC合并PSC患者UC多先于PSC确诊,多因肝功能异常发现PSC;主要临床表现为黄疸、腹痛、腹泻不适,部分可伴有发热、呕吐、乏力等症状;多有AKP升高、全结肠炎、直肠豁免、倒灌性回肠炎的临床特点;易合并胆管癌。

Objective To summarize the clinical characteristics of ulcerative colitis(UC)with primary sclerosing cholangitis(PSC).Methods The clinical characteristics,diagnosis and treatment of 6 UC patients with PSC admitted to Sir Run Run Shaw Hospital affiliated to Zhejiang University School of Medicine from May 2017 to May 2022 were analyzed retrospectively.Meanwhile“ulcerative colitis”,“primary sclerosing colangitis”were used as retrieval words to search associated literatures from CNKI,Wanfang Data,and PubMed databases as of August 2022.Relevant literatures were reviewed and summarized.Results There were 6 UC patients complicated with PSC,included 4 males and 2 females.Four UC cases were diagnosed before PSC and other 2 cases were diagnosed simultaneously.Six cases were all extensive colonic type(E3),2 patients were rectal sparing,and 4 patients had backwash ileitis.Five patients had elevated alkaline phosphatase(AKP)levels.All the patients received oral treatment with ursodeoxycholic acid(UDCA).Two patients with concurrent cholangiocarcinoma died within one year of follow⁃up.A total of 75 relevant literatures were retrieved.Conclusions Patients with UC complicated with PSC are often diagnosed with UC before PSC,and PSC is often discovered due to abnormal liver function.The main clinical manifestations include jaundice,abdominal pain and diarrhea,and some may be accompanied by symptoms such as fever,vomiting,and fatigue.There are clinical characteristics such as elevated AKP,pancolitis,rectal sparing,and backwash ileitis.UC complicated with PSC is more likely to develop cholangiocarcinoma.