Mortality and Cancer in Pediatric-onset IBD
Mortality and Cancer in Pediatric-onset IBD
Objectives Although the incidence of pediatric inflammatory bowel disease (IBD) continues to rise in Northern France, the risks of death and cancer in this population have not been characterized.
Methods All patients <17 years, recorded in EPIMAD registry, and diagnosed between 1988 and 2004 with Crohn's disease (CD) or ulcerative colitis (UC) were included. The observed incidences of death and cancer were compared with those expected in the regional general population obtained by French Statistical Institute (INSEE) and the cancer Registry from Lille. Comparisons were performed using Fisher's exact test and were expressed using the standardized mortality ratios (SMRs) and standardized incidence ratios.
Results A total of 698 patients (538 with CD and 160 with UC) were identified; 360 (52%) were men, the median age at IBD diagnosis was 14 years (12–16) and the median follow-up time was 11.5 years (7–15). During follow-up, the mortality rate was 0.84% (6/698) and did not differ from that in the reference population (SMR=1.4 (0.5–3.0); P=0.27). After a median follow-up of 15 years (10–17), 1.3% of patients (9/698) had a cancer: colon (n=2), biliary tract (cholangiocarcinoma; n=1), uterine cervix (n=1), prepuce (n=1), skin (basal cell carcinoma (n=2), hematological (acute leukemia; n=1), and small bowel carcinoid (n=1). There was a significantly increased risk of cancer regardless of gender and age (standardized incidence ratio=3.0 (1.3–5.9); P<0.02). Four out of nine patients who developed a cancer had received immunosuppressants or anti-tumor necrosis factor-α therapy (including combination therapy in three patients).
conclusions In this large pediatric population-based IBD cohort, mortality did not differ from that of the general population but there was a significant threefold increased risk of neoplasia.
Inflammatory bowel diseases (IBDs), Crohn's disease (CD), and ulcerative colitis (UC) are chronic disorders of unknown etiology with a potentially severe disease course. The incidence and prevalence of IBD are increasing in children. Pediatric-onset IBD is often characterized by widespread localization with a rapidly progressive disease course leading to early and frequent use of immunosuppressive therapies (thiopurines and methotrexate) (immunosuppressants, ISs) and biologics. In this context, data on mortality and cancer risk are important because they would allow patients and their families to be aware of prognosis and make informed decisions about treatment. Meta-analyses of population-based cohort studies have revealed standardized mortality ratios (SMRs) of 1.1 (95% confidence interval (CI), 0.9–1.2) in UC and 1.4 (95% CI, 1.3–1.5) in CD and a number of studies in adults have reported increased rates of colon carcinoma and extra-colonic cancer manifestations including skin cancer and lymphoma in IBD patients. However, little data are available in the context of pediatric-onset IBD. In a recent Danish cohort, patients diagnosed with IBD at a young age had a higher relative mortality rate than patients diagnosed at later ages, most likely due to an excess long-term risk of dying. Even so, the absolute risk was not high.
The aim of this study was to estimate the mortality and cancer risk in patients with pediatric-onset IBD by utilizing a large IBD population-based cohort from Northern France in comparison with a reference population. We also report, in detail, the clinical history of pediatric-onset IBD patients who died and/or developed cancer.
Abstract and Introduction
Abstract
Objectives Although the incidence of pediatric inflammatory bowel disease (IBD) continues to rise in Northern France, the risks of death and cancer in this population have not been characterized.
Methods All patients <17 years, recorded in EPIMAD registry, and diagnosed between 1988 and 2004 with Crohn's disease (CD) or ulcerative colitis (UC) were included. The observed incidences of death and cancer were compared with those expected in the regional general population obtained by French Statistical Institute (INSEE) and the cancer Registry from Lille. Comparisons were performed using Fisher's exact test and were expressed using the standardized mortality ratios (SMRs) and standardized incidence ratios.
Results A total of 698 patients (538 with CD and 160 with UC) were identified; 360 (52%) were men, the median age at IBD diagnosis was 14 years (12–16) and the median follow-up time was 11.5 years (7–15). During follow-up, the mortality rate was 0.84% (6/698) and did not differ from that in the reference population (SMR=1.4 (0.5–3.0); P=0.27). After a median follow-up of 15 years (10–17), 1.3% of patients (9/698) had a cancer: colon (n=2), biliary tract (cholangiocarcinoma; n=1), uterine cervix (n=1), prepuce (n=1), skin (basal cell carcinoma (n=2), hematological (acute leukemia; n=1), and small bowel carcinoid (n=1). There was a significantly increased risk of cancer regardless of gender and age (standardized incidence ratio=3.0 (1.3–5.9); P<0.02). Four out of nine patients who developed a cancer had received immunosuppressants or anti-tumor necrosis factor-α therapy (including combination therapy in three patients).
conclusions In this large pediatric population-based IBD cohort, mortality did not differ from that of the general population but there was a significant threefold increased risk of neoplasia.
Introduction
Inflammatory bowel diseases (IBDs), Crohn's disease (CD), and ulcerative colitis (UC) are chronic disorders of unknown etiology with a potentially severe disease course. The incidence and prevalence of IBD are increasing in children. Pediatric-onset IBD is often characterized by widespread localization with a rapidly progressive disease course leading to early and frequent use of immunosuppressive therapies (thiopurines and methotrexate) (immunosuppressants, ISs) and biologics. In this context, data on mortality and cancer risk are important because they would allow patients and their families to be aware of prognosis and make informed decisions about treatment. Meta-analyses of population-based cohort studies have revealed standardized mortality ratios (SMRs) of 1.1 (95% confidence interval (CI), 0.9–1.2) in UC and 1.4 (95% CI, 1.3–1.5) in CD and a number of studies in adults have reported increased rates of colon carcinoma and extra-colonic cancer manifestations including skin cancer and lymphoma in IBD patients. However, little data are available in the context of pediatric-onset IBD. In a recent Danish cohort, patients diagnosed with IBD at a young age had a higher relative mortality rate than patients diagnosed at later ages, most likely due to an excess long-term risk of dying. Even so, the absolute risk was not high.
The aim of this study was to estimate the mortality and cancer risk in patients with pediatric-onset IBD by utilizing a large IBD population-based cohort from Northern France in comparison with a reference population. We also report, in detail, the clinical history of pediatric-onset IBD patients who died and/or developed cancer.
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