Clinical Pattern of Early-Onset Inflammatory Bowel Disease in Saudi Arabia: A Multicenter National Study

Abstract

Background: The objectives of this multicenter national study were to compare the clinical phenotype of early-onset inflammatory bowel disease (IBD) (EO-IBD) with IBD in older children and to examine whether there is any variability in consanguinity rate and familial aggregation in EO-IBD compared with later onset IBD.

Methods: A retrospective analysis was performed on children aged 0 to 14 years with IBD in 17 centers located in geographically distinct regions in Saudi Arabia, from 2003 to 2012. Data of patients with EO-IBD (0 to <6 yrs) were compared with those with later onset IBD (6-14 yrs). Moreover, we evaluated differences in clinical pattern of infantile or toddler onset IBD subgroup (0-3 yr) as compared with those presenting in older children.

Results: Of 352 IBD patients identified during the 10-year study period, 76 children (21.6%) younger than 6 years were diagnosed with IBD. Among the Crohn's disease (CD) group, infantile or toddler onset CD subgroup showed a more frequent isolated colonic involvement (L2) than later-onset group (57% versus 20%; P = 0.002). Positive family history was significantly more common in the infantile or toddler onset ulcerative colitis subgroup (29.4% versus 4.2% in later onset ulcerative colitis; P < 0.0001). The consanguinity rate was significantly higher in the infantile or toddler onset CD subgroup as compared with later onset CD group (57.1% versus 25.3%; P = 0.04).

Conclusions: In conclusion, EO-IBD exhibits a unique clinical phenotype with a strikingly higher familial aggregation in early-onset ulcerative colitis. Our data suggest a significant genetic impact on the onset of CD in the very young children.

FIGURE 1.

Distribution of UC and CD cases based on the age of onset.

FIGURE 2.

A, Disease location at the diagnosis according to Paris classification in EO-UC (0 to <6 yr) and LO-UC (6–14 yr). E1, proctitis; E2, left-sided colitis; E3, extensive colitis; E4, pancolitis. B, Disease location at the diagnosis according to Paris classification in EO-CD (0 to <6 yr) and LO-CD (6–14 yr). L1, ileum; L2, colon; L3, ileocolon; L4, upper gastrointestinal.