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Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease: Prevalence, Presentation, and Anti-TNF
Thomas Greuter1, Fabio Bertoldo, Roman Rechner
1*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.
Insights
Extraintestinal manifestations (EIM) are common in pediatric inflammatory bowel disease (IBD), often appearing before diagnosis. Anti-tumor necrosis factor (TNF) therapy shows effectiveness for certain EIM in children with IBD.
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Rheumatology
Background:
- Limited data exists on extraintestinal manifestations (EIM) and their treatments in pediatric inflammatory bowel disease (IBD).
- Extraintestinal manifestations are common in pediatric IBD patients, with some appearing before diagnosis.
Purpose of the Study:
- To investigate the prevalence, timing, and treatment of EIM in a pediatric IBD cohort.
- To assess the effectiveness of anti-tumor necrosis factor (TNF) therapy for EIM in pediatric IBD.
Main Methods:
- Retrospective analysis of data from the Pediatric Swiss IBD Cohort Study (since 2008).
- Inclusion of 329 pediatric patients diagnosed with IBD (Crohn disease, ulcerative colitis, or IBD-unclassified).
- Evaluation of EIM occurrence, timing relative to IBD diagnosis, and treatment with anti-TNF agents.
Main Results:
- 16.7% of pediatric IBD patients experienced EIM, more frequently in Crohn disease than ulcerative colitis/IBD-unclassified.
- Peripheral arthritis and aphthous stomatitis were the most common EIM.
- 27.6% of EIM preceded the IBD diagnosis; 56.4% of patients with EIM received anti-TNF therapy, a higher proportion than those without EIM.
- Anti-TNF therapy showed response rates of 61.5% for peripheral arthritis and 66.7% for uveitis.
Conclusions:
- EIM are frequently observed in pediatric IBD, with a significant proportion appearing before diagnosis, potentially aiding earlier IBD detection.
- Anti-TNF agents are effective for treating specific EIM in pediatric IBD, but new EIM can still emerge during therapy.
Background:
There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD).
Methods:
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.
Results:
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%).
Conclusions:
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.
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