J Korean Med Sci.  2021 Nov;36(44):e278. 10.3346/jkms.2021.36.e278.

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

• Jang HJ
• Suh HR
• Choi S
• Hong SJ
• Cho SM
• Choi KH
• Choe BH
• Kang B
• on behalf of Crohn's and Colitis Association in Daegu-Gyeongbuk (CCAiD)

• KMID: 2522358
• DOI: http://doi.org/10.3346/jkms.2021.36.e278

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.

Keyword

Extraintestinal Manifestation; Crohn's Disease; Ulcerative Colitis; Pediatric; Paris Classification

Figure

• 

  Fig. 1 PUCAI scores in patients with UC. (A) Comparison between EIM-negative and -positive group. (B) Comparison between patients with S0 and S1 disease activity based on the Paris classification.PUCAI = Pediatric Ulcerative Colitis Activity Index, UC = ulcerative colitis, EIM = extraintestinal manifestation, S0 = never severe (PUCAI < 65), S1 = ever severe (PUCAI ≥ 65).

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