Phenotype and Disease Course of Early-onset Pediatric Inflammatory Bowel Disease

Abstract

BACKGROUND:\ud Early-onset (EO) pediatric inflammatory bowel diseases (IBD) seem to be more extensive than those with a later onset. To test this hypothesis, we examined the phenotype and disease course of patients with IBD diagnosis at 0 to 5 years, compared with the ranges 6 to 11 and 12 to 18 years.\ud METHODS:\ud Anatomic locations and behaviors were assessed according to Paris classification in 506 consecutive patients: 224 Crohn's disease, 245 ulcerative colitis, and 37 IBD-unclassified.\ud RESULTS:\ud Eleven percent of patients were in the range 0 to 5 years, 39% in 6 to 11 years, and 50% in 12 to 18 years. Ulcerative colitis was the most frequent diagnosis in EO-IBD and in 6- to 11-year-old group, whereas Crohn's disease was predominant in older children. A classification as IBD-unclassified was more common in the range 0 to 5 years compared with the other groups (P < 0.005). EO Crohn's disease showed a more frequent isolated colonic (P < 0.005) and upper gastrointestinal involvement than later-onset disease. Sixty-two percent of the patients in the 0 to 5 years range had pancolonic ulcerative colitis, compared with 38% of 6 to 11 years (P = 0.02) and 31% of 12-18 years (P = 0.002) range. No statistical difference for family history for IBD was found in the 3-year age groups. Therapies at the diagnosis were similar for all children. However, at latest follow-up, a significantly higher proportion of younger children were under steroids compared with older groups (P < 0.05). Surgical risk did not differ according to age.\ud CONCLUSIONS:\ud EO-IBD exhibits an extensive phenotype and benefit from aggressive treatment strategies, although surgical risk is similar to later-onset disease. A family history for IBD is not common in EO disease

Incidence and Phenotype at Diagnosis of Very-early-onset Compared with Later-onset Paediatric Inflammatory Bowel Disease: A Population-based Study [1988–2011]

“…In those with CD, the rate of complicated behaviours [B2 or B3] was similar in both age groups in our study, which contrasts with Gupta et al's finding of a higher rate of complicated behaviours in those with IBD aged 6-16 years/ 17 Upper gastrointestinal location [L4] and anoperineal lesions were found to be similar in both age groups. The rate of L4 among those with VEO-CD [32%] was similar to that reported by Aloi et al 15 but much higher than that found by Heyman et al [5%]. 7 In those with UC, the rates of ulcerative proctitis [E1] and extensive colonic involvement [E3/E4] were similar in both age groups.…”

“…Moreover, the time between the onset of symptoms and diagnosis was not delayed in the VEO-IBD group, whatever the type of IBD. As previously reported, 7,14,15,17,29,35 the initial presentation was different according to age group, with mucous bloody stools significantly more frequent in cases of VEO-IBD, probably because of the higher rate of isolated colonic disease in those with CD and a higher proportion of UC compared with older children. As noted by Gupta et al, 17 weight loss and abdominal pain were significantly more common in the EO-IBD group and this was also the case in our study.…”

“…However, CD represented 60% of IBD cases in our VEO-IBD group, unlike previous studies that reported a predominance of UC over CD in VEO-IBD. 14,15,29,32 This could have arisen from the diagnostic criteria used, as well as from specific environmental factors and lifestyle in the study area. 33,34 Complete bowel investigations were obtained as often in the VEO-IBD group as in the EO-IBD group and the diagnostic criteria [definite or probable IBD cases] did not change over time.…”

“…The cut-off of 6 years was chosen based on previous studies. 7,14,15,17 The EPIMAD Registry is a prospective population-based study recording all cases of IBD documented since 1988 in Northern France [ Figure 1]. This study area includes 5 864 508 inhabitants, representing 9.3% of the total French population, and is divided into four administrative areas.…”

“…6,7 A small number of monogenic mutations [8][9][10][11] have been identified in children with IBD diagnosis at a very young age, but genome-wide association studies failed to detect large differences between adult-onset and paediatric-onset disease. 12,13 Several studies reported different disease phenotypes in children with a diagnosis of IBD before 10 years of age compared with children aged over 10 years, 5,14,15 adolescents, or adults, leading to the Paris modification of the IBD Montreal classification, differentiating children with a diagnosis made before 10 years of age [A1a] from those with a diagnosis at 10-17 years [A1b]. 16 Thus, age at diagnosis is important clinically and it appears that very-early-onset IBD [VEO-IBD] [age < 6 years at diagnosis] might be a distinct form.…”