Very early onset inflammatory bowel disease in a South Asian country where inflammatory bowel disease is emerging: a distinct clinical phenotype from later onset disease

Abstract

Background/aims: Information on pediatric inflammatory bowel disease (PIBD) and very early onset IBD (VEOIBD) are sparse in India, where IBD is emerging. We aimed to evaluate characteristics of VEOIBD and later onset PIBD (LO-PIBD) in India.

Methods: We performed retrospective analysis of a large, prospectively maintained IBD registry. PIBD was divided in to VEOIBD ( < 6 years) and LO-PIBD (6-17 years). Demographic data, disease characteristics and treatment were compared between the PIBD groups and with other Asian/Western studies as well as the adult patients of the registry.

Results: Of 3,752 IBD patients, 292 (7.8%) had PIBD (0-17 years) (175 Crohn's disease [CD], 113 ulcerative colitis [UC], 4 IBD-undifferentiated; 22 VEOIBD [7.5%], and 270 LO-PIBD [92.5%]). VEOIBD patients had more severe disease compared to LO-PIBD in both UC (P= 0.003) and CD (P< 0.001). Familial IBD was more common in VEOIBD (13.6%) compared to LO-PIBD (9.2%). Ileal disease (L1) was an independent risk factor for diagnostic delay in pediatric CD. Diagnostic delay ( > 6 months) was significantly lower in VEOIBD (40.9%) than in LO-PIBD (78.8%) (P< 0.001). Compared to other Asian and Western studies, extensive UC (72.5%) and complicated CD (stricturing/penetrating: 42.7%) were relatively more common. Perianal CD was relatively less frequent (7.4%). PIBD had a significantly higher number of complicated and ileal CD and extensive UC comparison to adult cohort of the registry.

Conclusions: VEOIBD has more aggressive phenotype than LO-PIBD. Disease appears distinct from other Asian and Western studies and adult onset disease, with more complicated CD and extensive UC.

Keywords: Crohn disease; Inflammatory bowel diseases; Later onset pediatric IBD; Ulcerative colitis; Very early onset IBD.

Fig. 1.

Age distribution, disease extent, and phenotype of PIBD. (A) Distribution of UC and CD cases according to age groups. (B) Extent of disease in UC: VEOIBD and LO-PIBD patients. (C) Extent of disease in CD: VEOIBD and LO-PIBD patients. (D) Disease behavior in CD: VEOIBD and LO-PIBD patients. IBD, inflammatory bowel disease; CD, Crohn’s disease; UC, ulcerative colitis; IBD-U, undifferentiated IBD; VEOIBD, very early onset IBD; LO-PIBD, later onset pediatric IBD; E1, proctitis; E2, upto splenic flexure; E3, upto hepatic flexure; E4, pancolitis; L1, ileal; L2, colonic; L3, ileo-colonic; L4a, upper gastrointestinal disease upto ligament of Treitz; L4b, beyond ligament of Treitz upto distal ileum CD; B1, inflammatory; B2, stricturing disease; B3, fistulizing CD; p, perianal disease.