Greuter Thomas, Bertoldo Fabio, Rechner Roman, Straumann Alex, Biedermann Luc, Zeitz Jonas, Misselwitz Benjamin, Scharl Michael, Rogler Gerhard, Safroneeva Ekaterina, Ali Raja A R, Braegger Christian, Heyland Klaas, Mueller Pascal, Nydegger Andreas, Petit Laetitia-Marie, Schibli Susanne, Furlano Raoul I, Spalinger Johannes, Schäppi Michela, Zamora Samuel, Froehlich Florian, Herzog Denise, Schoepfer Alain M, Vavricka Stephan R
*Division of Gastroenterology and Hepatology, University Hospital Zurich †Division of Gastroenterology and Hepatology, Triemli Hospital Zurich, Zurich ‡Praxis Römerhof, Olten §Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland ||Division of Gastroenterology, The National University of Malaysia Medical Centre, Kuala Lumpur, Malaysia ¶Division of Gastroenterology and Nutrition and Children's Research Center, University Children's Hospital Zurich, Zurich #Department of Pediatrics, Kantonsspital Winterthur, Winterthur **Division of Gastroenterology and Hepatology, Kinderspital St Gallen, St Gallen ††Division of Pediatric Gastroenterology and Hepatology, Department of Pediatrics, University Hospital Lausanne-CHUV, Lausanne ‡‡Department of Pediatrics, University Hospital Geneva-HCUG, Geneva §§Division of Gastroenterology and Hepatology, University Children's Hospital Bern, Bern ||||Division of Gastroenterology and Nutrition, University Children's Hospital Basel-UKBB, Basel ¶¶Department of Pediatrics, Kantonsspital Luzern-LUKS, Luzern ##Private Practice, Pediatric Unit, Clinique des Grangettes, Geneva ***Private Practice, Onex †††Division of Gastroenterology and Hepatology, University Hospital Basel, Basel ‡‡‡Division of Gastroenterology and Hepatology, University Hospital Lausanne-CHUV, Lausanne §§§Department of Pediatrics, Hopital Cantonal de Fribourg, Fribourg, Switzerland.
J Pediatr Gastroenterol Nutr. 2017 Aug;65(2):200-206. doi: 10.1097/MPG.0000000000001455.
There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD).
Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively.
A total of 55 patients (16.7%) experienced 1-4 EIM (39 Crohn disease, 12 ulcerative colitis, and 4 IBD-unclassified patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs 5.0%, P = 0.014). EIM were more frequent in Crohn disease when compared to ulcerative colitis/IBD-unclassified (22.5% vs 10.3%, P = 0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). Approximately 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (-37.5-149.0). Thirty-one of the 55 patients (56.4%) were treated with 1 or more anti-tumor necrosis factor (TNF) agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs 35.0%, P = 0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%).
In a cohort of pediatric patients with IBD, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings may translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti-TNF for the treatment of certain EIM is effective, although a substantial proportion of new EIM may present despite ongoing anti-TNF therapy.
关于炎症性肠病（IBD）患儿肠外表现（EIM）及其治疗的数据匮乏。
自2008年以来，瑞士儿科IBD队列研究收集了瑞士儿科IBD人群的数据。对329例患者的数据进行回顾性分析。
共有55例患者（16.7%）出现1 - 4种EIM（39例克罗恩病、12例溃疡性结肠炎和4例未分类IBD患者）。在IBD发病时，EIM的出现比成人人群更频繁（8.5%对5.0%，P = 0.014）。与溃疡性结肠炎/未分类IBD相比，克罗恩病中EIM更常见（22.5%对10.3%，P = 0.003）。最常见的EIM是外周关节炎（26/329，7.9%）和口疮性口炎（24/329，7.3%）。所有EIM中约27.6%在IBD诊断之前出现。IBD诊断与首次出现EIM之间的中位时间为1个月（-37.5 - 149.0）。55例患者中有31例（56.4%）接受了1种或更多种抗肿瘤坏死因子（TNF）药物治疗。与没有EIM的IBD患者相比，有EIM的患者更有可能接受抗TNF治疗（56.4%对35.0%，P = 0.003）。对抗TNF的反应率取决于潜在的EIM，对外周关节炎（61.5%）和葡萄膜炎（66.7%）效果最佳。
在一组儿科IBD患者中，经常遇到EIM。高达30%的EIM在IBD诊断之前出现。了解这些发现可能会提高对潜在IBD的认识，从而减少诊断延迟。抗TNF治疗某些EIM是有效的，尽管在持续抗TNF治疗期间可能仍会出现相当比例的新EIM。
