P285. Cancer and mortality survey in paediatric patients with inflammatory bowel disease
L. de Ridder1, D. Turner2, S. Koletzko3, J. Martín de Carpi4, U.L. Fagerberg5, C. Spray6, M. Sladek7, D.C. Wilson8, E. Roma-Giannikou9, J. Bronsky10, D.E. Serban11, S. Cucchiara12, G. Veres13, F.M. Ruemmele14, I. Hojsak15, K.L. Kolho16, A. Levine17, 1Erasmus MC/Sophia Children's Hospital, Peadiatric Gastroenterology, Rotterdam, Netherlands, 2Shaare Zedek Medical Center, Jerusalem, Israel, 3University of Munich Medical Centre, Germany, 4Unidad para el Cuidado Integral de la Enfermedad Inflamatoria Intestinal Pediátrica, Sección de Gastroenterología, Hepatología y Nutrición Pediatrica, Hospital Sant Joan de Déu, Spain, 5Centre for Clinical Research, Västmanland County Hospital, Västerås, Karolinska Institutet, Sweden, 6Bristol Royal Hospital for Children, United Kingdom, 7Department of Pediatrics, Gastroenterology and Nutrition, Polish-American Children's Hospital, Jagiellonnian University Medical College, Poland, 8Child Life and Health, University of Edinburgh, United Kingdom, 91st Department of Paediatric of Athens University, Greece, 102nd Faculty of Medicine, Charles University and University Hospital Motol, Czech Republic, 11University of Medicine and Pharmacy Iuliu Hatieganu, Cluj-Napoca, Romania, 12Pediatric Gastroenterology and Liver Unit, “La Sapienza” University, Italy, 13Semmelweis University, Hungary, 14Université Paris Descartes, Sorbonne Paris Cité, APHP, Hôpital Necker Enfants Malades, France, 15Referral Center for Pediatric Gastroenterology, Children's Hospital Zagreb, School of Medicine, University of Zagreb, Croatia, 16Children's Hospital, Helsinki, University Central Hospital and University of Helsinki, Finland, 17Wolfson Medical Center, Israel
Incidence of paediatric inflammatory bowel disease (PIBD) has risen significantly in European countries during the past two decades. The combination of the typical severe phenotype of PIBD and the intensified medical treatment may be associated with increased risk of malignancy and mortality, but evidence is extremely scarce. The Porto IBD working group of ESPGHAN conducted a European survey of cancer and mortality in PIBD.
A survey among paediatric gastroenterologists of 19 European countries and Israel on cancer and/or mortality in the PIBD patient population was undertaken. One representative from each country repeatedly contacted all paediatric gastroenterologists from each country for reporting retrospectively PIBD patients (diagnosed before age 19 years) diagnosed with any cancer and/or mortality after diagnosis of IBD, during the period of 2006–2011.
We identified 44 cases (18 cancers and/or 32 deaths). Median age at diagnosis of IBD was 10.0 year (n = 26; 59% male). Type of IBD was Crohn's disease (n = 19; 43%), ulcerative colitis (n = 22; 50%) and IBD unclassified (n = 2; 5%). Causes of mortality were infectious (n = 15; 47%), uncontrolled disease activity of IBD (eg toxic megacolon) (n = 6; 19%), cancer (n = 6; 19%), other non-IBD related diseases (n = 3; 9%) and unknown (n = 2; 6%). The most common malignancy was hematopoetic tumors (n = 11; 61%), of which 3 were hepatosplenic T cell lymphoma and 2 EBV-associated lymphomas. Medications used in the three months preceding the mortality cases included steroids (n = 19, 59%), thiopurines (n = 18, 56%), biologics (n = 8, 25%) and calcineurin inhibitors (n = 7, 22%). Combination therapy (as defined as thiopurines and biologics) was used in five (16%). Medications used in the three months preceding the cancer cases included steroids (n = 4, 22%), thiopurines (n = 12, 67%), biologics (n = 2, 11%) and calcineurin inhibitors (n = 1, 6%). Combination therapy was used in only one patient (6%).
Cancer and mortality in PIBD are rare but the cumulative rates are not insignificant. Mortality is primarily related to infections. Uncontrolled disease activity and cancer were both responsible for 19% of deaths. The lack of a control group makes it impossible to elucidate how many of the cancer cases are disease-specific but at least five lymphomas were likely treatment-associated, by virtue of their phenotype. A minority of patients had been treated with combination therapy.