BACKGROUND AND AIMS: Ulcerative proctitis [UP] is an uncommon presentation in paediatric patients with ulcerative colitis. We aimed to characterize the clinical features and natural history of UP in children, and to identify predictors of poor outcomes. METHODS: This was a retrospective study involving 37 sites affiliated with the IBD Porto Group of ESPGHAN. Data were collected from patients aged <18 years diagnosed with UP between January 1, 2016 and December 31, 2020. RESULTS: We identified 196 patients with UP (median age at diagnosis 14.6 years [interquartile range, IQR 12.5-16.0]), with a median follow-up of 2.7 years [IQR 1.7-3.8]. The most common presenting symptoms were bloody stools [95%], abdominal pain [61%] and diarrhoea [47%]. At diagnosis, the median paediatric ulcerative colitis activity index [PUCAI] score was 25 [IQR 20-35], but most patients exhibited moderate-severe endoscopic inflammation. By the end of induction, 5-aminosalicylic acid administration orally, topically or both resulted in clinical remission rates of 48%, 48%, and 73%, respectively. The rates of treatment escalation to biologics at 1, 3, and 5 years were 10%, 22%, and 43%, respectively. In multivariate analysis, the PUCAI score at diagnosis was significantly associated with initiation of systemic steroids, or biologics, and subsequent acute severe colitis events and inflammatory bowel disease-associated admission, with a score ≥35 providing an increased risk for poor outcomes. By the end of follow-up, 3.1% of patients underwent colectomy. Patients with UP that experienced proximal disease progression during follow-up [48%] had significantly higher rates of a caecal patch at diagnosis and higher PUCAI score by the end of induction, compared to those without progression. CONCLUSION: Paediatric patients with UP exhibit high rates of treatment escalation and proximal disease extension.

2nd Clinic of Pediatrics 'Iuliu Hatieganu' University of Medicine and Pharmacy Emergency Clinical Hospital for Children Cluj Napoca Romania

Barts Health NHS Trust The Royal London Children's Hospital Department of Paediatric Gastroenterology Queen Mary University of London Centre for Immunobiology Blizard Institute London UK

Children's Health Ireland UCD and RCSI Dublin Ireland

Children's Hospital University of Helsinki Helsinki Finland

Department NEUROFARBA University of Florence Meyer Children's Hospital Florence Italy

Department of Clinical Science and Education Södersjukhuset Karolinska Institutet Stockholm Sweden

Department of Gastroenterology Hepatology and Nutrition Astrid Lindgren Children's Hospital Karolinska University Hospital StockholmSweden

Department of Paediatric Gastroenterology and Nutrition Kidz Health Castle UZ Brussels Free University Brussels Brussels Belgium

Department of Paediatric Gastroenterology and Nutrition Royal Hospital for Children and Young People Edinburgh UK

Department of Paediatric Gastroenterology Leeds Children's Hospital Leeds Teaching Hospitals NHS Trust Leeds UK

Department of Pediatric Gastroenterology Centro Materno Infantil do Norte Centro Hospitalar e Universitário de Porto Porto Portugal

Department of Pediatric Gastroenterology Great North Children's Hospital Newcastle UK

Department of Pediatric Gastroenterology Hepatology and Nutrition Hospital Sant Joan de Déu Barcelona Spain

Department of Pediatrics 2nd Medical Faculty Charles University and University Hospital Motol Prague Czech Republic

Department of Pediatrics Hvidovre University Hospital Copenhagen Hvidovre Denmark

Department of Pediatrics School of Medicine Kyungpook National University Daegu Korea

Department of Pediatrics St Marien Hospital Bonn Germany

Department of Translational Medical Science Section of Pediatrics University of Naples 'Federico II' Naples Italy

Division of Pediatric Gastroenterology and Nutrition Edmond and Lily Safra Children's Hospital Ramat Gan Israel

Faculty of Medicine and Health Technology University of Tampere Tampere Finland

Faculty of Medicine Tel Aviv University Tel Aviv Israel

Gastroenterology and Nutrition Department Hospital Infantil Universitario Niño Jesús Madrid Spain

Heim Pál National Pediatric Institute Budapest Hungary

Institute for Maternal and Child Health IRCCS 'Burlo Garofolo' Trieste Italy

Institute of Gastroenterology Nutrition and Liver Disease Schneider Children's Medical Center Petah Tikva Israel

Paediatric Gastroenterology Department Al Jalila Children's Specialty Hospital Mohammed Bin Rashid University Dubai United Arab Emirates

Pdiatric Gastroenterology Kaplan Medical Centre Rehovot and the Faculty of Medicine Hebrew University of Jerusalem Jerusalem Israel

Pediatric Gastroenterology and Cystic Fibrosis Unit Department of Human Pathology in Adulthood and Childhood 'G Barresi' University of Messina Messina Italy

Pediatric Gastroenterology and Liver Unit University Children's Hospital of the University Medical Centre Ljubljana Ljubljana Slovenia

Pediatric Gastroenterology and Nutrition Institute Ruth Children's Hospital of Haifa Rambam Medical Center Faculty of Medicine Technion Haifa Israel

Pediatric Gastroenterology Hepatology and Nutrition Institute Sapienza University of Rome Rome Italy

Pediatric Gastroenterology Institute 'Dana Dwek' Children's Hospital Tel Aviv Sourasky Medical Center Tel Aviv Israel

Pediatric Gastroenterology Unit Emek Medical Center Afula Israel

Pediatric Gastroenterology Unit Maggiore Hospital Bologna Italy

Pediatric Gastroenterology Unit Meir Medical Center Kfar Saba Israel

Pediatric Hepatology Gastroenterology and Transplantation Unit ASST Papa Giovanni XXIII Bergamo Italy

Referral Center for Pediatric Gastroenterology and Nutrition Children's Hospital Zagreb Zagreb Croatia

Sachs' Children and Youth Hospital Department of Gastroenterology Södersjukhuset Stockholm Sweden

Service de Gastroentérologie et Nutrition Pédiatriques Hôpital Universitaire Robert Debré Paris France

The Juliet Keidan Institute of Pediatric Gastroenterology and Nutrition Shaare Zedek Medical Center The Hebrew University of Jerusalem Jerusalem Israel

The Ruth and Bruce Rappaport Faculty of Medicine Technion Israel Institute of Technology Haifa Israel

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