Front. Pediatr., 31 May 2022, Sec. Pediatric Gastroenterology, Hepatology and Nutrition
Volume 10 – 2022 | https://doi.org/10.3389/fped.2022.8759

Abstract

Introduction: Pediatric patients with inflammatory bowel disease (IBD) are classified into Crohn’s disease (CD), ulcerative colitis (UC), and unclassifiable (IBD-U). However, data provide evidence that ileal CD (L1) is distinct from colonic CD (L2). The aim of this study was to investigate the clinical features of isolated Crohn’s colitis in a pediatric population.

Material and Methods: Children who were prospectively included in the CEDATA–GPGE registry on diagnosis were compared according to the diagnosis of CD with L2 vs. L1 and ileocolonic (L3) involvement pattern as well as IBD-U and UC. The clinical significance of L2 was investigated with regard to extraintestinal manifestations, treatment, surgery, and disease activity.

Results: Fifty-two patients with L2 CD at a median age of 13.4 years (±3.8 SD) were compared with 182 L1 (13.8 ± 2.9 SD), 782 with L3 (12.8 ± 3.3 SD), 653 with UC (12.7 ± 3.8 SD), and 111 patients with IBD-U (11.9 ± 4.7 SD). Bloody stools at diagnosis were more common in L2 (44%) than in L1 (19.7%) and L3 (28.8%), but not as common as in UC (66.5%) and IBD-U (61.3%). Fewer CD patients with L2 (10.2%) received exclusive enteral nutrition therapy (EEN) as induction than patients with L1 (34.3%) and L3 (33.3%). After induction therapy, 42.3% of patients with L2 received immunosuppressants and 21% biologicals during follow-up (L1 56.5/10.5%; L3 59/21%; CU 43.5/11.9%; IBD-U 26.1/12.6%). Extraintestinal manifestations were more frequent in L2 (23.1%) vs. L1 (18.7%), L3 (20.2%), CU (15.8%), and IBD-U (11.7%). The number of patients requiring surgery did not differ within the CD subgroups and was significantly lower in UC and IBD-U. Perianal fistula surgery was significantly more common in L2 (44%) than in L1 (4.8%) or L3 (21.7%). In addition, the frequency of surgery for perianal abscesses was also more frequent in L2 (55.6%) than in L1 (12.7%) or L3 (38.4%).

Conclusions: The consideration of pediatric Crohn’s colitis as a distinct disease seems necessary as it is characterized by extraintestinal manifestations (EIMs) with mainly joint involvement and perianal fistulas or abscesses requiring surgery and biologic therapy. Thus, colonic Crohn’s disease may have an influence on the therapeutic stratification and should be addressed in further studies.

Copyright © 2022 Elonen, Wölfle, de Laffolie, Posovszky and the CEDATA–GPGE-Study-Group. 

Der Beitrag Isolated Crohn’s Colitis: Is Localization Crucial? Characteristics of Pediatric Patients From the CEDATA–GPGE Registry erschien zuerst auf CEDATA GPGE – Patientenregister.

Front Med (Lausanne) . 2021 Aug 27;8:644003. doi: 10.3389/fmed.2021.644003. eCollection 2021.

• PMCID: PMC8430211
• DOI: 10.3389/fmed.2021.644003

Abstract

Background and Aims: Intensifying therapy for Paediatric Crohn’s Disease (CD) by early use of immunomodulators and biologics has been proposed for cases in which predictors of poor outcome (POPO) were present. We investigated therapy stratifying potential comparing POPO-positive and -negative CD patients from CEDATA-GPGE®, a German-Austrian Registry for Paediatric Inflammatory Bowel disease. 

Methods: CD patients (1-18 years) registered in CEDATA-GPGE® (2004-2018) within 3 months of diagnosis and at least two follow-up visits were included. Disease course and treatments over time were analysed regarding positivity of POPO criteria and test statistical properties. 

Results: 709/1084 patients included had at least one POPO criterion (65.4%): 177 patients (16.3%) had persistent disease (POPO2), 581 (53.6%) extensive disease (POPO3), 21 (1.9%) severe growth retardation POPO4, 47 (4.3%) stricturing/penetrating disease (POPO6) and 122 (11.3%) perianal disease (POPO7). Patients with persistent disease differed significantly in lack of sustained remission >1 year (Odd Ratio (OR) 1.49 [1.07-2.07], p = 0.02), patients with initial growth failure in growth failure at end of observation (OR 51.16 [19.89-131.62], p < 0.0001), patients with stricturing and penetrating disease as well as perianal disease in need for surgery (OR 17.76 [9.39-33.58], p < 0.001; OR 2.56 [1.58-4.15], p < 0.001, respectively). Positive Predictive Value for lack of sustained remission was >60% for patients with initial growth failure, persistent or stricturing/penetrating disease. 

Conclusion: Predictors of poor outcome with complicated courses of disease were common in CEDATA-GPGE®. An early intensified approach for paediatric CD patients with POPO-positivity (POPO2-4, 6-7) should be considered, because they have an increased risk to fare poorly.

Der Beitrag Running Behind „POPO“ – Impact of Predictors of Poor Outcome for Treatment Stratification in Pediatric Crohn’s Disease erschien zuerst auf CEDATA GPGE – Patientenregister.

DATA FROM THE CEDATA GPGE REGISTRY

To date, failure of the first-line medical management of Crohn’s disease (CD) in children and adolescents has been followed by escalation treatment, with administration of immunomodulators, e.g., azathioprine, or biologics, such as infliximab (1). The most recent revision of the international guideline proposes stratification of the therapy, with selection of either accelerated step-up or primary treatment with biologics on the basis of predictors of poor outcome (POPO) (2). The objective of this study was to evaluate this concept of early immunosuppression with the aid of data from the largest registry of children and adolescents with chronic inflammatory bowel disease (IBD) in Europe.

Der Beitrag Early Immunosuppression in Children and Adolescents with Crohn’s Disease erschien zuerst auf CEDATA GPGE – Patientenregister.

• PMID: 28604511
• DOI: 10.1097/MPG.0000000000001649

Abstract

Objectives: Perianal disease (PD) with fistula and/or abscess formation is a severe complication in Crohn disease (CD). We examined prevalence, incidence, and risk factors for PD development in a pediatric CD cohort.

Methods: Patients with CD from the prospective, multicenter registry for inflammatory bowel disease from Germany and Austria (CEDATA-GPGE) were included if diagnosed at the age of 18 years or younger, registered within 3 months after diagnosis, and having at least 2 follow-up visits within the first year of registration. We examined potential risk factors for PD with Kaplan-Meier analysis and a final Cox model considering sex, family history of inflammatory bowel disease, extraintestinal manifestations, disease location, and induction therapy (corticosteroids or nutritional therapy).

Results: Of 2406 patients with CD, 742 fulfilled inclusion criteria (59% boys, mean age at diagnosis 12.4 ± 3.4 years). PD was present at diagnosis in 41 patients (5.5%; 80.9% boys), whereas 32 patients (4.3%, 81.3% male) developed PD during follow-up (mean 2.0 ± 1.6 years). The cumulative incidence of PD at 12 and 36 months after diagnosis was 3.5% and 7.5%, respectively. Potential risk factors for PD development during follow-up were male sex (hazard ratio = 3.2, [95%; confidence interval 1.2-7.8]) and induction therapy with corticosteroids (hazard ratio = 2.5 [1.1-5.5]). Diagnostic evaluation at PD diagnosis was incomplete in 40% of affected subjects. PD resolved within 1 year in 50% of cases.

Conclusions: Approximately 10% of CD patients in our cohort suffered from PD within the first 3 years of their disease. Male sex and initial corticosteroid therapy were associated with an increased risk to develop PD after diagnosis.

Der Beitrag Incidence and Risk Factors for Perianal Disease in Pediatric Crohn Disease Patients Followed in CEDATA-GPGE Registry erschien zuerst auf CEDATA GPGE – Patientenregister.