Abstract
Abstract Background Concurrent immune-mediated extraintestinal manifestations (IM-EIMs; Table 1) are common in paediatric-onset inflammatory bowel disease (pIBD), but the overall epidemiology is unknown. This systematic review and meta-analysis aimed to summarise the prevalence and management of EIMs in pIBD. Methods MEDLINE, EMBASE and CENTRAL, clinicaltrials.gov and conference abstracts were searched up to May 6, 2024, for studies that included pIBD (2-17 years old) with secondarily diagnosed EIMs. The primary outcome included the prevalence of IM-EIMs among pIBD. Secondary outcomes included efficacy and adverse effects of treatment on EIMs. Meta-analysis included pooled proportions with DerSimonian-Laird random-effects analysis. Results The pooled proportion of IM-EIMs among pIBD was 8.4% (95% CI, 6.5-11.0%; I2=97%) among 53 eligible studies. The pooled proportion of IM-EIMs was 10.3% (95% CI, 7.3-13.8%; I2=95%) among 37 Crohn’s disease (CD) studies, compared to 11.3% (95% CI, 7.8-15.3%; I2=93%) among 39 ulcerative colitis (UC) studies. Enthesitis was the highest reported individual IM-EIM (23%, 95% CI, 4-50%; I2=94.7%) in pIBD. Arthritis was the second highest reported individual IM-EIM (8%, 95% CI, 6-10%; I2=96%) in pIBD, followed by primary sclerosing cholangitis (PSC) in UC (5%, 95% CI, 4-7%; I2=81%; Table 2). Uveitis, pyoderma gangrenosum, autoimmune hepatitis, and PSC in CD were the lowest reported IM-EIMs at 1% or less. The largest difference in reported proportions between IBD phenotypes was PSC, which was 4% higher in UC than CD. Only one eligible study described treatment response. Conclusion This meta-analysis identified that while musculoskeletal manifestations are relatively common, fewer than 5% of pIBD patients experience ophthalmological, dermatological, and hepatic IM-EIMs. There was significant methodological and statistical heterogeneity as expected for the relatively small number of individual paediatric studies and likely resulted in the large difference of pooled proportions between individual IM-EIMs. Multicentre collaborative efforts are needed to systematically describe the epidemiology and management outcomes of EIMs in pIBD in the current era of advanced therapies.
Published Version
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have