Inflamm Bowel Dis. 2023 Nov 23:izad275. doi: 10.1093/ibd/izad275. Online ahead of print.

Manuela Distante ¹, Silvia Rotulo ¹, Marco Ranalli ¹, Eugenio Pedace ¹, Paolo Lionetti ², Serena Arrigo ³, Patrizia Alvisi ⁴, Erasmo Miele ⁵, Massimo Martinelli ⁵, Giovanna Zuin ⁶, Matteo Bramuzzo ⁷, Mara Cananzi ⁸, Marina Aloi ¹; SIGENP IBD Working Group

Collaborators,

SIGENP IBD Working Group: M Baldi, C Banzato, G Barera, G Castellucci, M Corpino, R Cozzali, C De Giacomo, P Diaferia, D Dilillo, E Felici, M T Illiceto, D Knafelz, L Norsa, P M Pavanello, A Ravelli, V Romagnoli, C Romano, S Salvatore

Author information

¹Pediatric Gastroenterology and Liver Unit, Department of Maternal and Child Health, Umberto I Hospital, Sapienza University of Rome, Rome, Italy.

²Unit of Gastroenterology and Nutrition, Meyer Children's Hospital, Florence, Italy.

³Gastroenterologia ed Endoscopia Pediatrica, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

⁴Pediatric Gastroenterology Unit, Maggiore Hospital, Bologna, Italy.

⁵Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples, Italy.

⁶Pediatrics, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.

⁷Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy.

⁸Unit of Gastroenterology, Digestive Endoscopy, Hepatology and Care of Children with Liver Transplantation, University Hospital of Padova, Padova, Italy.

Abstract

Background: This study aimed to define clusters of disease activity and prognostic factors of disease course in a well-characterized cohort of children with Crohn's disease (CD).

Methods: All patients from the SIGENP IBD (Italian Society of Pediatric Gastroenterology Hepatology and Nutrition Inflammatory Bowel Disease) registry with a 5-year follow-up and 6-monthly evaluation were included. Active disease was defined for each semester as follows: clinical activity (weighted Pediatric Crohn's Disease Activity Index ≥12.5 or Mucosal Inflammation Noninvasive Index ≥8) and active disease on endoscopy (Simple Endoscopic Score for Crohn's Disease >3 or fecal calprotectin >250 µg/g) or imaging. Formula-based clusters were generated based on previously published patterns in adults.

Results: Data from 332 patients were analyzed. A total of 105 (32%) experienced a quiescent disease course; 49 (15%) and 31 (9%) a moderate-to-severe chronically active and chronic intermittent disease, respectively; 104 (31%) and 43 (13%) had active disease in the first 2 years after diagnosis and remission thereafter and vice versa, respectively. Surgery at diagnosis was significantly associated with a quiescent course (odds ratio [OR], 10.05; 95% confidence interval [CI], 3.05-25.22; P=.0005), while growth impairment at the diagnosis and active disease requiring corticosteroids at 6 months were inversely related to the quiescent group (OR, 0.48; 95% CI, 0.27-0.81; P= .007; and OR, 0.35; 95% CI, 0.16-0.71; P= .005, respectively). Perianal involvement at diagnosis and moderate-severe activity at 6 months correlated with disease progression (OR, 3.85; 95% CI, 1.20-12.85; P=.02).

Conclusions: During the first 5 years of follow-up, one-third of children with CD experience a quiescent course. However, another one-third have a moderate-to-severe disease course. Surgery at the diagnosis is related to a quiescent course, while growth impairment and lack of response to induction therapy correlate with more severe disease activity during follow-up.