Severe Disease Activity Based on the Paris Classification Is Associated with the Development of Extraintestinal Manifestations in Korean Children and Adolescents with Ulcerative Colitis

Abstract

Background There are limited data regarding the extraintestinal manifestations (EIMs) associated with pediatric inflammatory bowel disease (IBD) in Korea. We aimed to investigate the clinical features and factors associated with the development of EIMs in Korean children and adolescents with IBD. Methods This multicenter, retrospective study was conducted from 2010 to 2017. Baseline clinicodemographic, laboratory findings, disease activity, disease phenotypes, and EIMs were investigated. Results A total of 172 patients were included. One-hundred thirty-seven (79.7%) had Crohn's disease (CD), and 35 (20.3%) had ulcerative colitis (UC). EIMs occurred in 42 patients (24.4%). EIMs developed in 34/137 diagnosed with CD (24.8%), and in 8/35 diagnosed with UC (22.9%), during a median follow-up duration of 3.2 (interquartile range, 1.9–5.4) years for CD and 3.0 (1.0–4.0) years for UC, respectively. Arthritis/arthralgia was most commonly observed (n = 15, 35.7%), followed by stomatitis/oral ulcer (n = 10, 23.8%), hepatitis (n = 5, 11.9%), nephritis (n = 4, 9.5%), pancreatitis (n = 2, 4.8%), erythema nodosum (n = 2, 4.8%), pyoderma gangrenosum (n = 1, 2.4%), primary sclerosing cholangitis (n = 1, 2.4%), uveitis (n = 1, 2.4%), and ankylosing spondylitis (n = 1, 2.4%). A significant difference in disease severity based on the Paris classification ( P = 0.011) and ESR at diagnosis ( P = 0.043) was observed between the EIM positive and negative group in patients with UC. According to logistic regression analyses, S1 disease severity based on the Paris classification was the only factor that was significantly associated with the development of EIMs (odds ratio, 16.57; 95% confidence interval, 2.18–287.39; P = 0.017). Conclusion Severe disease activity based on the Paris classification in pediatric patients with UC was significantly associated with EIM development. As disease severity in the Paris classification is a dynamic parameter, treatment should be focused on disease control to minimize the occurrence of EIMs in Korean children and adolescents with UC.

The evaluation of extraintestinal manifestations in children diagnosed with inflammatory bowel disease: A single-center experience

“…However, contrary to these findings, PUCAI and PCDAI scores at diagnosis did not differ significantly between EIM-positive and EIM-negative patients. [10] Regression analysis also revealed no significant relationship between the development of EIM and PUCAI. [10] Yu et al [6] found a relationship between the severity of the disease at diagnosis and the development of EIM.…”

“…It was found to be the most common EIM joint finding (arthritis) in IBD patients (12-21%). [6,10,12,13,16,17] In a study designed by Rahmani et al [12] in CD and UC patient groups, the incidence of EIM related with joint was found to be 21.2% and 12.5%, respectively. Oral aphtha was seen in 15% of CD patients, but not in UC patients.…”

“…The incidence of EIM in IBD patients is around 11-28%. [6,10,13] EIMs are more common in women than men. [15] CD (14-24%) patients have more EIM than UC (11-22%) patients.…”

“…[9] Although EIMs are known to be common in patients with pediatric-onset IBD, few studies have examined EIM rates and their association with other disease-related factors. [10] Therefore, in our study, we aimed to examine the relationship between the disease activity index rates at the time of diagnosis and the development of EIM in children and adolescents with IBD.…”

Vedolizumab Is Safe and Efficacious for the Treatment of Pediatric-Onset Inflammatory Bowel Disease Patients Who Fail a Primary Biologic Agent

“… 1 Once IBD is diagnosed in children, the goals of therapy consist of eliminating symptoms, normalizing quality of life, restoring growth, and preventing complications while minimizing the adverse effects of medications. 1 2 In addition, pediatric IBD has a more aggressive disease course than adult-onset disease. Therefore, early introduction of immunomodulators including anti-tumor necrosis factor (TNF) agents is required.…”