Department of Gastroenterology, College of Medicine, Sir Run Run Shaw Hospital, Zhejiang University, Hangzhou, 310016, China.
Inflammatory Bowel Disease Center, College of Medicine, Sir Run Run Shaw Hospital, Zhejiang University, Hangzhou, 310016, China.
Int J Colorectal Dis. 2024 Jul 13;39(1):107. doi: 10.1007/s00384-024-04688-7.
Family history is one of the strongest risk factors for inflammatory bowel diseases (IBD) while studies about the clinical phenotype of familial IBD are limited. This study aimed to compare the phenotypic features of familial Crohn's disease (CD) with sporadic CD.
Familial CD was defined as CD patients having one or more first, second, third, fourth degree, or above relatives with CD. Sporadic CD patients hospitalized during the same period were matched 1:3 by age and gender. Differences in clinical characteristics, phenotype distribution, extraintestinal manifestations, and complications at diagnosis, as well as treatment regimen and surgery, were compared between familial and sporadic CD.
The familial CD was associated with a higher rate of past appendectomy history (P = 0.009), more intestinal perforation at onset (P = 0.012), more MRI results of anal lesion (P = 0.023), and gastrointestinal perforation (P = 0.040) at diagnosis, higher rate of past intestinal surgery history (P = 0.007), more number of intestinal surgeries (P = 0.037), longer duration of follow-up (P = 0.017), lower rate of taking biologicals for current maintenance (P = 0.043), lower tendency to upgrade to biologicals during follow-up (P = 0.013), higher possibility to experience gastrointestinal obstruction (P = 0.047), and abdominal abscess during follow-up (P = 0.045).
Familial CD is associated with a more aggressive clinical phenotype.
家族史是炎症性肠病(IBD)最强的危险因素之一,而关于家族性 IBD 的临床表型的研究有限。本研究旨在比较家族性克罗恩病(CD)与散发性 CD 的表型特征。
家族性 CD 定义为 CD 患者有一个或多个一级、二级、三级、四级或以上亲属患有 CD。同期住院的散发性 CD 患者按年龄和性别 1:3 匹配。比较家族性和散发性 CD 之间在临床特征、表型分布、肠外表现和诊断时的并发症、治疗方案和手术方面的差异。
家族性 CD 与过去阑尾切除术史的发生率较高相关(P=0.009),发病时肠穿孔的发生率较高(P=0.012),MRI 结果肛门病变的发生率较高(P=0.023),诊断时胃肠道穿孔的发生率较高(P=0.040),过去肠手术史的发生率较高(P=0.007),肠手术次数较多(P=0.037),随访时间较长(P=0.017),当前维持治疗使用生物制剂的比例较低(P=0.043),随访期间升级为生物制剂的趋势较低(P=0.013),发生胃肠道梗阻的可能性较高(P=0.047),腹部脓肿的发生率较高(P=0.045)。
家族性 CD 与更具侵袭性的临床表型相关。