Kwon Yiyoung, Kim Eun Sil, Choe Yon Ho, Kim Mi Jin
Department of Pediatrics, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul, South Korea.
Front Pediatr. 2022 May 23;10:853019. doi: 10.3389/fped.2022.853019. eCollection 2022.
Ulcerative colitis (UC) is a systemic inflammatory disease with a gut predominance, which may involve other organs. The presence of extraintestinal manifestation (EIM) is an important symptom for clinicians as it alters the treatment decisions. In this study, we aimed to evaluate the initial clinical presentation and disease severity of pediatric UC patients with EIMs.
One hundred forty-two patients under the age of 18 years who were diagnosed with UC from January 2003 to November 2021 were included in this study. Forty-seven patients with confirmed EIMs and 95 patients without EIMs were divided into two groups and their differences were analyzed.
The most common EIM was peripheral arthritis. The disease extent at the time of diagnosis shows a higher rate of pancolitis in the EIM-positive group (65.9%) than that of the EIM-negative group (33.7%) ( < 0.001). More than 90% of EIM-positive patients had moderate to severe disease activity on the Mayo endoscopic subscore. In the EIM-positive group, the cumulative use of systemic steroids, immunosuppressants, and biological agents from diagnosis to 1 year follow-up were significantly higher than those of the EIM-negative group ( = 0.009, 0.001, and < 0.001, respectively). About 80% of patients in the EIM-negative group reached remission, but only about 50% of the EIM-positive patients reached remission ( = 0.005). The relapse occurred more frequently in the EIM-positive group than in the EIM-negative group with statistical significance ( < 0.001).
Pediatric UC with EIMs had higher disease severity and often manifested upper gastrointestinal tract involvement. Despite EIMs treatment, the occurrence of new EIMs was observed repeatedly. Cumulative drug demand (steroids, immunosuppressants, and biological agents) for the treatment increased steadily over time, and frequent relapses occurred despite the combinatory use of therapeutic drugs.
溃疡性结肠炎（UC）是一种以肠道为主的全身性炎症性疾病，可能累及其他器官。肠外表现（EIM）的出现是临床医生的一个重要症状，因为它会改变治疗决策。在本研究中，我们旨在评估患有EIM的儿科UC患者的初始临床表现和疾病严重程度。
本研究纳入了2003年1月至2021年11月期间诊断为UC的142例18岁以下患者。47例确诊为EIM的患者和95例无EIM的患者分为两组，并分析他们之间的差异。
最常见的EIM是外周关节炎。诊断时的疾病范围显示，EIM阳性组的全结肠炎发生率（65.9%）高于EIM阴性组（33.7%）（<0.001）。超过90%的EIM阳性患者在梅奥内镜亚评分上有中度至重度疾病活动。在EIM阳性组中，从诊断到1年随访期间全身类固醇、免疫抑制剂和生物制剂的累计使用量显著高于EIM阴性组（分别为=0.009、0.001和<0.001）。EIM阴性组约80%的患者达到缓解，但EIM阳性组只有约50%的患者达到缓解（=0.005）。EIM阳性组的复发比EIM阴性组更频繁，具有统计学意义（<0.001）。
患有EIM的儿科UC疾病严重程度更高，常表现为上消化道受累。尽管对EIM进行了治疗，但仍反复观察到新的EIM出现。治疗所需的累计药物（类固醇、免疫抑制剂和生物制剂）随着时间的推移稳步增加，尽管联合使用了治疗药物，但仍频繁复发。
