New ESPGHAN-ECCO Guidelines on Ulcerative Colitis in Children
P-ECCO Committee Member
The European Crohn’s and Colitis Organisation (ECCO) is pleased to announce the upcoming release of new guidelines on the management of Ulcerative Colitis (UC) in paediatric patients, developed in collaboration with the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN). This important document aims to provide up-to-date recommendations that reflect the latest advancements in research and clinical practice, ensuring optimal care for children suffering from UC.
The guidelines focus on both the ambulatory setting and the management of Acute Severe Colitis (ASC), serving as an update to the previous ESPGHAN-ECCO guidelines published in 2018 [1, 2].
Understanding Ulcerative Colitis in Children
As the global incidence of paediatric-onset UC has continued to rise [3], the burden of the disease and its impact on patients, families and society have grown [4]. Paediatric UC is generally more extensive and severe than adult-onset UC [5], and despite the recent introduction of advanced therapies [6], treatment options remain limited due to significant regulatory challenges in approving drugs for children [7]. Due to these unique characteristics, UC in children is more prone to acute severe exacerbations and failure of therapeutic regimens [8]. Together with emerging new evidence, these factors underscore the urgent need for updated guidelines to support physicians in managing paediatric UC.
Key Highlights of the Guidelines
Diagnosis and Assessment: Although the initial diagnostic algorithm [1] is not the primary focus of these guidelines, they emphasise a multidisciplinary approach to assessing UC in children, including clinical evaluation, endoscopy, imaging and histological assessment. The guidelines once more highlight the importance of the Paediatric Ulcerative Colitis Activity Index (PUCAI), which, according to several studies, can help predict the prognosis of children with UC both at diagnosis and after induction therapy [9]. ASC, as the most critical emergency in paediatric IBD, is defined by a PUCAI score (≥65), either at diagnosis or during a disease flare [10].
Guidelines acknowledge a growing trend toward close assessment of patient-reported outcomes (PROs), which are closely linked to a patient's quality of life [11]. Several studies have suggested that there can be a significant discrepancy between patients’ perceptions of their disease and symptom severity and the perceptions of their treating physician [12]. Therefore, PROs should be recognised as an important part of the investigation that can assist in patient engagement and empowerment.
Another addition is the use of colonic ultrasound to monitor disease activity in paediatric UC [13]. Two recent meta-analyses by the same research team, one conducted in healthy children [14] and the other in children with IBD [15], identified a bowel wall thickness cut-off of 2 mm as indicative of an inflamed colon.
Treatment Strategies: A major focus of the guidelines is the establishment of treatment plans. The recommendations advocate for the use of both pharmacological and non-pharmacological therapies, tailored to the severity of the disease and the specific needs of the patient.
As in previous guidelines [1], recommendations include a step-up approach, beginning with 5-aminosalicylic acid (5-ASA) as a first-line induction and maintenance therapy for mild-moderate UC cases and escalating to corticosteroids (for induction treatment) or immunomodulators for maintenance treatment if needed.
The absence of any recommendation for a universal top-down approach was justified by the fact that, in contrast to Crohn’s Disease, time to initiation of biologics is not associated with the risk of colectomy, as shown in population-based cohorts [16]. Based on the data in adults but also in children [17], infliximab should be considered, preferably in combination with an immunomodulator, as the first-line biologic agent in chronically active or corticosteroid-dependent UC uncontrolled by 5-ASA, and in most cases also thiopurines, for both induction and maintenance of remission. In most cases, higher doses of infliximab (e.g. 10 mg/kg/dose at weeks 0, 2 and 6, followed by 10 mg/kg every 4–8 weeks for maintenance) are required to provide the best chance of achieving the desired clinical and endoscopic outcome. Adalimumab, on the other hand, may be considered in those with immunogenic loss of response to infliximab, based on serum trough concentrations and antibodies, and can also be offered as a first-line biologic in selected non-severe cases. Proactive therapeutic drug monitoring is recommended for both infliximab and adalimumab, particularly at the end of induction.
Due to its good safety profile and established long-term use in children [18], vedolizumab has been recommended as a second-line biologic therapy for chronically active or corticosteroid-dependent patients who have failed anti-TNF treatment.
One large section of the guidelines focuses on novel therapeutic options. While most of the available evidence for the use of advanced treatments comes from adult studies, there is emerging data for paediatric patients as well [19–24]. In cases of anti-TNF failure, several other treatment options have been proposed, including anti-p40 agents (IL-12/23 inhibitors, e.g. ustekinumab), anti-p19 agents (IL-23 inhibitors, e.g. risankizumab, mirikizumab, guselkumab), JAK inhibitors (e.g. tofacitinib, upadacitinib, filgotinib), golimumab [25, 26] and S1P receptor agonists (e.g. ozanimod, etrasimod). The selection of the agent depends on many factors and should be made individually.
Treatment of Acute Severe Colitis: There is limited new evidence on the use of intravenous corticosteroids in ASC since publication of the previous guideline, so the recommendations remain largely unchanged [2]. Intravenous methylprednisolone continues to be the first-line treatment for ASC and should be started promptly. However, efforts should be made to shorten the duration of corticosteroid use if there is no response, as continuing corticosteroids beyond this point offers no additional benefit and increases the risk of toxicity. Second-line therapy should be started by the fifth day of intravenous corticosteroid treatment in children with a PUCAI ≥65, with infliximab remaining the preferred choice for second-line medical therapy. Calcineurin inhibitors (tacrolimus or cyclosporine) may be considered as an alternative second-line treatment in centres with expertise in their use, particularly after a previous failure of infliximab. For third-line treatment, sequential rescue therapies—such as calcineurin inhibitors following infliximab, infliximab following calcineurin inhibitors or a JAK inhibitor after either—may be considered for stable patients in specialised centres.
The updated guidelines reflect a change in the recommendation regarding venous thromboembolic events (VTEs), since while paediatric IBD patients have a lower incidence of VTEs compared to adult IBD patients, they still face an increased absolute risk. The incidence of VTEs in paediatric IBD patients ranges from 3.7 to 31.2 per 10,000 patient-years, which is higher than that among the general paediatric population, either hospitalised or ambulatory [27–29]. Therefore, pharmacological thromboprophylaxis for VTEs should be considered for all paediatric patients hospitalised with ASC.
Monitoring and Follow-up: Ongoing monitoring is crucial for the effective management of UC. The guidelines recommend regular assessments of disease activity, nutritional status, bone health and medication adherence to ensure that treatment remains effective and side effects are addressed promptly.
Although the recommendations focus on the paediatric population, the guidelines also include colorectal cancer (CRC) surveillance due to the increased risk of CRC in individuals with paediatric-onset UC. While only a few cases of dysplasia and CRC have been reported in UC patients under 18 years old [31, 32], the risk over time remains a concern. Children with UC aged 12 years and older, and with a disease duration of more than 8 years, should be considered for CRC and dysplasia surveillance. Additionally, those with concomitant primary sclerosing cholangitis should begin surveillance at age 12, regardless of disease duration.
Psychosocial Support: Recognising the emotional and psychological impact of chronic illness, the guidelines highlight that symptoms of anxiety, depression and poor quality of life are often linked to poorer treatment adherence, with non-adherence rates reaching up to 90% in adolescents with IBD [33]. Therefore, the guidelines stress the importance of providing psychosocial support to both patients and their families. This holistic approach emphasises that effective management of UC requires attention to mental health as well as the physical symptoms of the disease.
Conclusion
As the landscape of paediatric gastroenterology continues to evolve, the collaboration between ECCO and ESPGHAN underscores the commitment to improving outcomes for children with UC. These guidelines serve not only as a comprehensive resource for clinicians, but also as a critical tool for supporting all healthcare providers in managing the complexities associated with this chronic condition. We invite them all to access the full guidelines and to integrate these recommendations into their practice, with the goal of achieving better management of paediatric UC.
For more information, visit the ECCO Website or consult the full text of the guidelines when it becomes available through ESPGHAN.
References
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