Inflammatory bowel disease in pediatric patients: Characteristics of newly diagnosed patients from the CEDATA-GPGE Registry

Abstract

Background: Inflammatory bowel disease (IBD) can arise at any age, with peak incidence in adolescence and young adulthood. A registry of pediatric cases of IBD offers the opportunity to document their diagnosis and treatment, with the ultimate aim of improving diagnosis and treatment in the future.

Methods: In the German-language CEDATA-GPGE registry, 3991 cases of IBD in patients less than 18 years of age were documented from 2004 to 2014. The 1257 patients who were prospectively included in the registry upon diagnosis and whose further course was documented for at least three months were analyzed in two separate groups--under 10 years old, and 10 years and above--with respect to the type and duration of their symptoms until diagnosis, the completeness of the diagnostic evaluation, the disease phenotype, and the initial treatment.

Results: Of the 958 patients for whom full documentation was available, 616 (64.3%) had Crohn's disease (CD), 278 (29%) had ulcerative colitis (UC), 64 (6.7%) had an unclassified IBD, and 23.2% were under 10 years old. The latency to diagnosis was longer for CD than for UC (0.5 versus 0.3 years), regardless of age. 62.5% of the CD patients had ileocolonic involvement, and more than half had involvement of the upper gastrointestinal tract. 71% of the patients with UC had subtotal colitis or pancolitis. Continuous improvement was seen in diagnostic assessment according to published guidelines. For example, in 2004/2005, 69% of patients were evaluated endoscopically with ileocolonoscopy and esophagogastroduodenoscopy; this fraction had risen to nearly 100% by 2013/2014. Similarly, the percentage of patients who underwent a diagnostic evaluation of the small intestine, as recommended, rose from 41.2% to 60.9% over the same period. The most common initial treatments were 5- amino - salicylates (86.8% CD, 100% UC) and glucocorticoids (60.6% CD, 65.6% UC). 32% of the patients with CD received exclusive enteral nutrition therapy.

Conclusion: Most of these pediatric patients with IBD, whether in the younger or the older age group, had extensive bowel involvement at the time of diagnosis. The registry data imply that improvement in clinical course may be achieved by shortening the time to diagnosis and by closer adherence to the diagnostic and therapeutic guidelines.

Figure 1

Description of patient cohorts of CEDATA-GPGE registry. CD, Crohn’s disease; IBD, inflammatory bowel disease; UC, ulcerative colitis; IBD-U, unclassified inflammatory bowel disease; EGD, esophagogastroduodenoscopy

Figure 2

Diagnoses and sex distribution of 958 patients with new-onset inflammatory bowel disease CD, Crohn’s disease; UC, ulcerative colitis; IBD-U, unclassified inflammatory bowel disease

Figure 3

Increase in ileocolonoscopy and esophagogastroduodenoscopy rates at new diagnosis over the registry period from 2004 to 2014; confidence intervals

eFigure 1

CEDATA-GPGE initial reporting form

eFigure 2

CEDATA-GPGE reporting form

eFigure 3

Graphic representation of Paris classification of disease phenotype, ordered by location of affected portion of intestine

eFigure 4

Distribution of age at diagnosis of new inflammatory bowel disease (IBD) patients, by diagnosis. IBD-U, unclassified IBD

eFigure 5

Summary of cumulative performance of methods compliant with diagnostic guidelines (reference to all 958 patients) IBD, inflammatory bowel disease; EGD, esophagogastroduodenoscopy; MRI, magnetic resonance imaging