Isolated Crohn's Colitis: Is Localization Crucial? Characteristics of Pediatric Patients From the CEDATA–GPGE Registry

Lotta Elonen, Lena Wölfle, Jan de Laffolie, Carsten Posovszky, the CEDATA–GPGE-Study-Group, Tobias Schwerdt, Rainer Ganschow, Stefan Trenkel, Burkhard Rodeck, Stefan Wirth, Marlen Zurek, Matthias Heiduk, Michael Paulussen, Gunter Flemming, Ekkehard Sturm, Axel Enninger, Söhnke Dammann, Henning Böhme, Michael Melter, Thomas Lang, Philip Bufler, Thomas Lücke, Markus Knuf, Norbert Wagner, Thomas Kaiser, Ralf Pallacks, Andre Hörning, Jens Klinge, Steffen Reinsch, Rüdiger Adam, Stefan Buderus, Markus Richter, Antje Ballauf, Ilse Broekaert, Lars Heerdts, Carolin Blüml, Sabine Peitzsch, Andreas Krahl, Simone Jedwilayties, Maik Heine, Marko Reitmann, Kai Nils Pargac, Thomas Lang, Jutta Kringel, Anke Dick, Patrick Gerner, Michael Friedt, Enno Iven, Gunter Burmester, Anke Esser, Olaf Raecke, Kerstin Ehrentraut, Esther Schmidt, Jan Däbritz, Stefan Sgoll, Ahlke Willenborg, Sebastian Horn, Ralph Melchior, Rüdiger Kardoff, Martina Kohl-Sobania, Benedikt Pircher, Christoph Ehrsam, Daniela Nolkemper, Adrian Lieb, Almuth Hauer, Markus Prenninger, Martin Laaß, Dieter Furthner

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. 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. 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%). 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.