Clusters of Disease Activity and Early Risk Factors of Clinical Course of Pediatric Crohn's Disease

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

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


SIGENP IBD Working Group: M BaldiC BanzatoG BareraG CastellucciM CorpinoR CozzaliC De GiacomoP DiaferiaD DililloE FeliciM T IllicetoD KnafelzL NorsaP M PavanelloA RavelliV RomagnoliC RomanoS 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.


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.

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